{"id":3695,"date":"2019-07-21T13:08:15","date_gmt":"2019-07-21T11:08:15","guid":{"rendered":"https:\/\/aa-pnh.pe-dev.de\/diseases\/aplastic-anaemia\/"},"modified":"2026-04-04T16:09:07","modified_gmt":"2026-04-04T14:09:07","slug":"aplastic-anaemia","status":"publish","type":"page","link":"https:\/\/aa-pnh.org\/en\/diseases\/aplastic-anaemia\/","title":{"rendered":"Aplastic Anaemia"},"content":{"rendered":"<h2><span style=\"color: #ffcc00;\">APLASTIC ANAEMIA (AA)<\/span><\/h2>\n<p>&nbsp;<\/p>\n<input type=\"hidden\" bg_collapse_expand_pro=\"69e3e86a19db50091554226\" value=\"69e3e86a19db50091554226\"><input type=\"hidden\" id=\"bg-show-more-text-69e3e86a19db50091554226\" value=\"      1 What is AA?\"><input type=\"hidden\" id=\"bg-show-less-text-69e3e86a19db50091554226\" value=\"      1 What is AA?\"><input type=\"hidden\" id=\"bg-icon-bg-color-69e3e86a19db50091554226\" value=\"\"><a id=\"bg-showmore-action-69e3e86a19db50091554226\" class=\"bg-showmore-plg-link bg-arrow  \" style=\"color:#ffcc00;;font-weight:bold; font-size:24px; line-height: 150%\" href=\"#\">      1 What is AA?<\/a><ul class=\"bg-showmore-no-padding-no-margin bg-showmore-closed\" id=\"bg-showmore-pro-hidden-69e3e86a19db50091554226\">\n<h4><span style=\"color: #ffcc00;\">1.1 General<\/span><\/h4>\n<p>Aplastic anaemia is a non-<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;malignant&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Characterises abnormal cell growth&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/malignant\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">malignant<\/a> haematological disease. It is due to a disorder of bone marrow function that results in reduced development of blood cells.<br>\nAplastic anaemia can be divided into congenital forms (e.g. Diamond-Blackfan or <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;Fanconi anaemia&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Hereditary disease in which red and white blood cells are produced in a reduced quantity and broken down more quickly&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/fanconi-anaemia\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">Fanconi anaemia<\/a>) and acquired forms, depending on the age at which it occurs. Acquired forms can occur at any age. <\/p>\n<h4><span style=\"color: #ffcc00;\">1.2 Occurrence (epidemiology)<\/span><\/h4>\n<p>The <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;incidence&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Frequency of new cases per 100,000 inhabitants per year&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/incidence\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">incidence<\/a> of <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia in Central Europe is 2-3 new cases per million people per year. Aplastic anaemia is therefore a very rare disease. Most people who are affected fall ill between the ages of 10 and 25 or above the age of 60, with both sexes being equally affected.  <\/p>\n<h4><span style=\"color: #ffcc00;\">1.3 Origin (pathogenesis)<\/span><\/h4>\n<p>Studies have shown that a subtype of <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;lymphocyte&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Subgroup of white blood cells that play a role in the defence against diseases and foreign substances&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/lymphocyte\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">lymphocyte<\/a> in the body&rsquo;s own <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immune system&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Defence system; system that enables the body to fight off infections and to distinguish between its own and foreign tissues. In humans, the immune system consists of specialised proteins (antibodies), immune cells (white blood cells) and immune organs. It is responsible for our body&amp;#039;s defences(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immune-system\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immune system<\/a> attacks cells in the bone marrow, and this autoimmune process prevents the development of new blood cells.<br>\nIn most cases, it is not possible to identify the cause of <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia, so the origin of the disease is still unknown (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;idiopathic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Without identifiable cause&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/idiopathic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">idiopathic<\/a>). In some cases, medications, <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;toxic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;toxic&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/toxic\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>toxic<\/a> substances or viral infections are thought to be the cause. <\/p>\n<h4><a id=\"diakrit\"><\/a><span style=\"color: #ffcc00;\">1.4 Diagnostic criteria and classification<\/span><\/h4>\n<p><strong>In order to classify a disease as <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia, the following criteria must be met:<\/strong><\/p>\n<ul>\n<li>The number of cells in the bone marrow (cellularity) is less than 25% of that in healthy bone marrow, based on a <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;bone marrow biopsy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Taking a sample from the bone marrow (usually from the iliac crest) with a tissue punch. This method enables more precise examinations to be carried out than with bone marrow aspiration, since the bone punch cylinder is also obtained. &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/bone-marrow-biopsy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">bone marrow biopsy<\/a>. Cell production may be reduced during the course of the disease (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;hypoplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Mal\/underdevelopment of the bone marrow or an organ&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/hypoplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">hypoplastic<\/a>) or completely absent (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a>). <\/li>\n<li>Reduction of two (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;bicytopenia&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Lack of blood cells in two cell lines&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/bicytopenia\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">bicytopenia<\/a>) or three cell lines (tri- or <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;pancytopenia&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Tricytopenia; deficiency of blood cells of all three cell lines (erythrocytes, leukocytes, platelets)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/pancytopenia\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">pancytopenia<\/a>) of varying degrees in the blood film.<\/li>\n<li>There is no evidence of (new) development of connective tissue in the bone marrow (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;fibrosis&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Abnormal proliferation of connective tissue in the bone marrow or an organ&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/fibrosis\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">fibrosis<\/a>) or invasion of the bone marrow by <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;malignant&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Characterises abnormal cell growth&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/malignant\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">malignant<\/a> cells or cells outside the bone marrow.<\/li>\n<li>No radiotherapy or chemotherapy has been carried out recently that might explain a disorder of bone marrow function (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;bone marrow insufficiency&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Reduced production of blood cells in the bone marrow&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/bone-marrow-insufficiency\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">bone marrow insufficiency<\/a>).<\/li>\n<li>In addition, there have been no significant cell changes (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;dysplasia&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Malformation. Degenerated cells are &amp;quot;dysplastic&amp;quot;, i.e. malformed. Dysplastic cells do not mature (differentiate) normally.  &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/dysplasia\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">dysplasia<\/a>) in blood development (haematopoiesis).<\/li>\n<\/ul>\n<p>Aplastic anaemia is subdivided according to blood values (see table below) into:<\/p>\n<ul>\n<li><strong>non-severe<\/strong> <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia = nSAA (&ldquo;non-severe AA&rdquo;)<\/li>\n<li><strong>severe<\/strong> <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia = SAA (&ldquo;severe AA&rdquo;)<\/li>\n<li><strong>very severe<\/strong> <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia = vSAA (&ldquo;very severe AA&rdquo;)<\/li>\n<\/ul>\n<p>and is of crucial importance for prognosis and treatment.<\/p>\n<p><\/p><div id=\"attachment_1484\" style=\"width: 482px\" class=\"wp-caption alignnone\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-1484\" class=\"wp-image-1484\" src=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/2019\/07\/AA_Klassifikation_web-1.gif\" alt=\"Diagnosis of AA: Classification of Aplastic Anaemia\" width=\"472\" height=\"274\"\/><p id=\"caption-attachment-1484\" class=\"wp-caption-text\"><i>Classification of aplastic anaemia based on the blood count (cell count and film) Two out of three criteria must be met *For classification as vSAA, the criterion granulocytes &lt; 0,2 G\/l must be met.<\/i><\/p><\/div>\n<p><\/p><\/ul><ul style=\"margin-top:0;margin-bottom:0\"><style type=\"text\/css\">a.bg-showmore-plg-link:hover,a.bg-showmore-plg-link:active,a.bg-showmore-plg-link:focus{color:#0071bb;}<\/style>\n<input type=\"hidden\" bg_collapse_expand_pro=\"69e3e86a1a0000099502016\" value=\"69e3e86a1a0000099502016\"><input type=\"hidden\" id=\"bg-show-more-text-69e3e86a1a0000099502016\" value=\"      2 Symptoms\"><input type=\"hidden\" id=\"bg-show-less-text-69e3e86a1a0000099502016\" value=\"      2 Symptoms\"><input type=\"hidden\" id=\"bg-icon-bg-color-69e3e86a1a0000099502016\" value=\"\"><a id=\"bg-showmore-action-69e3e86a1a0000099502016\" class=\"bg-showmore-plg-link bg-arrow  \" style=\"color:#ffcc00;;font-weight:bold; font-size:24px; line-height: 150%\" href=\"#\">      2 Symptoms<\/a><\/ul><ul class=\"bg-showmore-no-padding-no-margin bg-showmore-closed\" id=\"bg-showmore-pro-hidden-69e3e86a1a0000099502016\">\n<h4><span style=\"color: #ffcc00;\">2.1 Anaemia<\/span><\/h4>\n<p>A reduction in oxygen-transporting red blood cells (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;erythrocyte&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Red blood cell; erythrocytes make up the majority of cellular blood components. They are formed in the bone marrow, contain haemoglobin (the red pigment in blood ) and have a life expectancy of about 120 days in healthy people. The haemoglobin content of the blood is an important measurement(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/erythrocyte\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">erythrocyte<\/a>s) can cause weakness, <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;fatigue&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Severe tiredness, exhaustion or increased need for rest; a symptom that often accompanies various chronic diseasesitet&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/fatigue\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">fatigue<\/a> and shortness of breath and even palpitations, especially during physical exertion. In addition, patients with anaemia often show paleness, especially in the palms of the hands, although the presence of paleness is not evidence of anaemia. <\/p>\n<h4><span style=\"color: #ffcc00;\">2.2 Increased susceptibility to infection<\/span><\/h4>\n<p>A reduced number of white blood cells (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;leukocytes&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;White blood cell. Cells with a wide variety of forms and functions. The leukocytes are responsible for defending against pathogens and removing debris due to decaying cells.  &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/leukocytes\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">leukocytes<\/a>) increases the risk of infection. Since the body&rsquo;s own defence system does not function sufficiently with a reduced number of <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;neutrophils&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Subgroup of granulocytes with an important role in defending against bacterial and fungal infections&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/neutrophils\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">neutrophils<\/a>, a subtype of white blood cells, such an infection can take a life-threatening course within hours and lead to blood poisoning (septicaemia).<br>\nIt is therefore important that you inform your doctor immediately if you develop a fever. A fever is defined as a body temperature of over 38&deg;C measured in the ear twice within an hour or over 38.3&deg;C measured once in the ear. <\/p>\n<h4><span style=\"color: #ffcc00;\">2.3 Bleeding<\/span><\/h4>\n<p>If the number of blood platelets (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;thrombocytes&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Smallest form of blood cells, whose main task is to maintain blood clotting&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/thrombocytes\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">thrombocytes<\/a>) is reduced, blood clotting may be impaired. This can lead to bleeding gums and <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;petechia&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;pl. petechiae; tiny, localised, red spot on the skin caused by bleeding from small blood vessels close to the skin. Petechiae are often caused by a lack of platelets. &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/petechia\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">petechia<\/a>e, small punctiform bleedings in the skin, or bruises (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;haematoma&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Bruise&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/haematoma\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">haematoma<\/a>s). These can also occur spontaneously, i.e. without previous injury. In the case of impaired clotting, even a relatively slight bleeding or injury (e.g. during a visit to the dentist) can be serious. In the event of bleeding, you should therefore contact your doctor as soon as possible so that he can decide whether special measures (e.g. platelet <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a>) are necessary.    <\/p>\n<p><\/p><\/ul><ul style=\"margin-top:0;margin-bottom:0\"><style type=\"text\/css\">a.bg-showmore-plg-link:hover,a.bg-showmore-plg-link:active,a.bg-showmore-plg-link:focus{color:#0071bb;}<\/style>\n<input type=\"hidden\" bg_collapse_expand_pro=\"69e3e86a1a0b04003214731\" value=\"69e3e86a1a0b04003214731\"><input type=\"hidden\" id=\"bg-show-more-text-69e3e86a1a0b04003214731\" value=\"      3 Diagnosis\"><input type=\"hidden\" id=\"bg-show-less-text-69e3e86a1a0b04003214731\" value=\"\"><input type=\"hidden\" id=\"bg-icon-bg-color-69e3e86a1a0b04003214731\" value=\"\"><a id=\"bg-showmore-action-69e3e86a1a0b04003214731\" class=\"bg-showmore-plg-link bg-arrow  \" style=\"color:#ffcc00;;font-weight:bold; font-size:24px; line-height: 150%\" href=\"#\">      3 Diagnosis<\/a><\/ul><ul class=\"bg-showmore-no-padding-no-margin bg-showmore-closed\" id=\"bg-showmore-pro-hidden-69e3e86a1a0b04003214731\">\n<p>If one or more of the above complaints and symptoms are present, the family doctor will have a blood test done. If this reveals an irregularity in the blood count, the patient may be referred to a specialist in haematology and\/or oncology.. <\/p>\n<p><strong>A number of further examinations will be carried out in this case:<\/strong><\/p>\n<ul>\n<li>Medical history (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;anamnesis&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Medical history, development of symptoms&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/anamnesis\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">anamnesis<\/a>), including the family history and a detailed record of any medications taken<\/li>\n<li>Physical examination, e.g. signs of anaemia or bleeding<\/li>\n<li>Cell studies\n<ul>\n<li style=\"list-style-type: none;\">\n<ul>\n<li>Microscopic <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;differential blood count&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Routine examination that indicates the cellular composition of the white blood cells (leukocytes) in the blood. The percentages of the individual blood cell types are determined by microscopic counting of a blood smear. The white cells of normal blood include granulocytes (neutrophils,(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/differential-blood-count\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">differential blood count<\/a><\/li>\n<li>Reticulocytes<\/li>\n<li>PNH diagnostics (a <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;PNH clone&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;All blood cells affected by the mutation typical of PNH; the size of the PNH clone is a measure of the severity of the disease&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/pnh-clone\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">PNH clone<\/a> is detectable in up to 70% of AA cases), see Chapter <a href=\"https:\/\/aa-pnh.org\/en\/diseases\/pnh\/\">4.3 PNH, 3 Diagnosis<\/a><\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/li>\n<li>Clinical chemistry\n<ul>\n<li style=\"list-style-type: none;\">\n<ul>\n<li>Haemolysis parameters: in particular <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;LDH&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;lactate dehydrogenase; marker in the blood indicating cell damage&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/ldh\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>LDH<\/a>, haptoglobin, bilirubin<\/li>\n<li>Coagulation: quick value, PTT, fibrinogen<\/li>\n<li>Liver function parameters: AST, ALT and AP<\/li>\n<li>Renal function parameters: <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;creatinine&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Nitrogenous metabolic end product that is excreted via the kidneys. An elevated creatinine level in the blood indicates impaired kidney function &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/creatinine\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">creatinine<\/a>, uric acid<\/li>\n<li>Blood sugar<\/li>\n<li>Total <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;protein&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Macromolecule consisting of one or more long chains of amino acids&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/protein\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">protein<\/a>, electrophoresis, immunoglobulins<\/li>\n<li>Inflammation parameters CRP<\/li>\n<li>Vitamin B12 and folic acid levels<\/li>\n<li>Iron status: <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ferritin&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Protein that stores iron; if ECs are given frequently, the ferritin level should be monitored in case chelation therapy becomes necessary.&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/ferritin\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">ferritin<\/a>. At <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ferritin&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Protein that stores iron; if ECs are given frequently, the ferritin level should be monitored in case chelation therapy becomes necessary.&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/ferritin\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">ferritin<\/a> values &gt; 1000 ng\/ml further clarification of possible organ damage due to possible iron overload<\/li>\n<li>Virus diagnostics: hepatitis A, B, C; HIV, EBV, CMV, Parvovirus B19<\/li>\n<li>Antinuclear and anti-DNA antibodies<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/li>\n<li>Functional diagnostics\n<ul>\n<li style=\"list-style-type: none;\">\n<ul>\n<li>Ultrasound (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;sonography&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Ultrasound; imaging method of examination&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/sonography\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">sonography<\/a>) of heart and upper abdomen<\/li>\n<li>X-ray examination of the chest (<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;thorax&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Chest, thorax&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/thorax\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>thorax<\/a>)<\/li>\n<li>ECG<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/li>\n<li>Special investigations<\/li>\n<li style=\"list-style-type: none;\">\n<ul>\n<li style=\"list-style-type: none;\">\n<ul>\n<li><a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA typing&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Examination of HLA characteristics A, B, C, DRB1 and DQB1 for stem cell transplantation&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla-typing\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">HLA typing<\/a> of the patient and his siblings<\/li>\n<li>In cases of suspected &ldquo;congenital&rdquo; <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;bone marrow insufficiency&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Reduced production of blood cells in the bone marrow&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/bone-marrow-insufficiency\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">bone marrow insufficiency<\/a> syndrome, further diagnostic tests, e.g. chromosome breakage analysis in case of suspected <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;Fanconi anaemia&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Hereditary disease in which red and white blood cells are produced in a reduced quantity and broken down more quickly&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/fanconi-anaemia\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">Fanconi anaemia<\/a>, <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;telomere&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;End of chromosomes that shorten with each cell division&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/telomere\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">telomere<\/a> length determination if <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;telomeropathy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Disease in which telomere shortening is exacerbated&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/telomeropathy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">telomeropathy<\/a> is suspected, genetic tests<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p>If a reduced number of one or more blood cell lines is confirmed without any known cause for increased damage or breakdown of these blood cells, an urgent bone marrow examination should be carried out. This will help to determine whether there is a disorder of blood development or another causeegt. <\/p>\n<p>For this purpose, a <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;bone marrow puncture&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Piercing through the bone into the bone marrow with a hollow needle in order to remove tissue for examination. Usually performed at the iliac crest, it can be used for bone marrow aspiration or bone marrow biopsy.&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/bone-marrow-puncture\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">bone marrow puncture<\/a> is performed, which can be done on an outpatient basis. A bone cylinder is usually taken from the pelvis under local anaesthetic using a hollow needle (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;Jamshidi needle&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Punch needle for bone marrow biopsy&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/jamshidi-needle\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">Jamshidi needle<\/a>) (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;bone marrow biopsy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Taking a sample from the bone marrow (usually from the iliac crest) with a tissue punch. This method enables more precise examinations to be carried out than with bone marrow aspiration, since the bone punch cylinder is also obtained. &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/bone-marrow-biopsy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">bone marrow biopsy<\/a>, bone marrow punch). This cylinder is approx. 1.5 cm in length with a diameter of 2-3 mm and is examined and evaluated under a microscope (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;histology&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Science of tissues: the branch of medicine that involves tissues at the microscopic level&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/histology\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">histology<\/a>).  <\/p>\n<p>In addition, blood, bone marrow and yellow marrow fragments are obtained with <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;bone marrow aspiration&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suction of cells and marrow fragments from the bone marrow (usually from the iliac crest) with a hollow needle&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/bone-marrow-aspiration\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">bone marrow aspiration<\/a>. These are spread out over a glass microscope slide, dried and stained. They are then assessed under the microscope, whole and in their position in relation to each other (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;cytological&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;The field of general biology that covers the structure and function of cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/cytological\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">cytological<\/a> examination).<\/p>\n<p>Furthermore, genetic tests can be carried out on the bone marrow cells, and the results of these tests can help to distinguish between diagnoses.<\/p>\n<p>As the individual laboratory steps in preparing the bone marrow <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;histology&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Science of tissues: the branch of medicine that involves tissues at the microscopic level&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/histology\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">histology<\/a> are time-consuming, it takes about 1-2 weeks to obtain a complete result. If the development of two or three cell lines (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;erythrocyte&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Red blood cell; erythrocytes make up the majority of cellular blood components. They are formed in the bone marrow, contain haemoglobin (the red pigment in blood ) and have a life expectancy of about 120 days in healthy people. The haemoglobin content of the blood is an important measurement(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/erythrocyte\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">erythrocyte<\/a>s, <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;leukocytes&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;White blood cell. Cells with a wide variety of forms and functions. The leukocytes are responsible for defending against pathogens and removing debris due to decaying cells.  &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/leukocytes\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">leukocytes<\/a>, platelets) is impaired according to the <span style=\"color: #000080;\"><a style=\"color: #000080;\" href=\"#diakrit\">diagnostic criteria<\/a><\/span> in Section 1.4 without the presence of pathologically altered cells (e.g. leukaemia cells) and without prior chemotherapy or radiotherapy, the disease is referred to as <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia<\/p>\n<p>&nbsp;<\/p>\n<p><\/p><div id=\"attachment_3510\" style=\"width: 361px\" class=\"wp-caption alignnone\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-3510\" class=\"wp-image-3510 size-full\" src=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/2019\/07\/AA_KM_apl_ges_web-1-e1563705277717.gif\" alt=\"Diagnosis of AA: Healthy and aplastic bone marrow\" width=\"351\" height=\"226\"\/><p id=\"caption-attachment-3510\" class=\"wp-caption-text\"><i>Bone marrow in a patient with aplastic anaemia compared with healthy bone marrow. In the diseased bone marrow one can see mostly connective tissue and fat cells. In the healthy marrow, the blood cells stand out from the large white fat cells as small coloured dots.  <\/i><\/p><\/div>\n<p>&nbsp;<\/p>\n<p><strong>The aim of these numerous investigations is to:<\/strong><\/p>\n<ul>\n<li>Exclude other diseases<\/li>\n<li>Discover the possible causes (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aetiology&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Cause of a disease&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aetiology\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aetiology<\/a>)<\/li>\n<li>Determine the severity of the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia<\/li>\n<li>Determine the prognosis<\/li>\n<\/ul>\n<p>In patients with severe or very severe <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia who are in good physical condition, it is advisable to carry out <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA typing&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Examination of HLA characteristics A, B, C, DRB1 and DQB1 for stem cell transplantation&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla-typing\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">HLA typing<\/a> of the patient as soon as the condition has been diagnosed. If the patient has siblings, they can also be typed to determine their suitability for stem cell donation. <\/p>\n<p><\/p><\/ul><ul style=\"margin-top:0;margin-bottom:0\"><style type=\"text\/css\">a.bg-showmore-plg-link:hover,a.bg-showmore-plg-link:active,a.bg-showmore-plg-link:focus{color:#0071bb;}<\/style>\n<input type=\"hidden\" bg_collapse_expand_pro=\"69e3e86a1a21f0083165993\" value=\"69e3e86a1a21f0083165993\"><input type=\"hidden\" id=\"bg-show-more-text-69e3e86a1a21f0083165993\" value=\"      4 Clinical course\"><input type=\"hidden\" id=\"bg-show-less-text-69e3e86a1a21f0083165993\" value=\"      4 Clinical course\"><input type=\"hidden\" id=\"bg-icon-bg-color-69e3e86a1a21f0083165993\" value=\"\"><a id=\"bg-showmore-action-69e3e86a1a21f0083165993\" class=\"bg-showmore-plg-link bg-arrow  \" style=\"color:#ffcc00;;font-weight:bold; font-size:24px; line-height: 150%\" href=\"#\">      4 Clinical course<\/a><\/ul><ul class=\"bg-showmore-no-padding-no-margin bg-showmore-closed\" id=\"bg-showmore-pro-hidden-69e3e86a1a21f0083165993\">\n<p>Without specific therapy, <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia is fatal in up to 70% of cases in adulthood.<\/p>\n<p>Aplastic anaemia may develop into myelodysplastic syndrome (MDS) or <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;acute&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Sudden onset, rapidly progressing&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/acute\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>acute<\/a> myeloid leukaemia (AML). In addition, some AA patients have a PNH-specific <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;mutation&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Alteration of genetic material (gene)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/mutation\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">mutation<\/a>.<\/p>\n<p><\/p><\/ul><ul style=\"margin-top:0;margin-bottom:0\"><style type=\"text\/css\">a.bg-showmore-plg-link:hover,a.bg-showmore-plg-link:active,a.bg-showmore-plg-link:focus{color:#0071bb;}<\/style>\n<input type=\"hidden\" bg_collapse_expand_pro=\"69e3e86a1a2d34043614719\" value=\"69e3e86a1a2d34043614719\"><input type=\"hidden\" id=\"bg-show-more-text-69e3e86a1a2d34043614719\" value=\"      5 Treatment\"><input type=\"hidden\" id=\"bg-show-less-text-69e3e86a1a2d34043614719\" value=\"      5 Treatment\"><input type=\"hidden\" id=\"bg-icon-bg-color-69e3e86a1a2d34043614719\" value=\"\"><a id=\"bg-showmore-action-69e3e86a1a2d34043614719\" class=\"bg-showmore-plg-link bg-arrow  \" style=\"color:#ffcc00;;font-weight:bold; font-size:24px; line-height: 150%\" href=\"#\">      5 Treatment<\/a><\/ul><ul class=\"bg-showmore-no-padding-no-margin bg-showmore-closed\" id=\"bg-showmore-pro-hidden-69e3e86a1a2d34043614719\">\n<h4><span style=\"color: #ffcc00;\">5.1 Overview<\/span><\/h4>\n<p>Haematological spontaneous healing (spontaneous <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;remission&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Temporary alleviation or cessation of the symptoms of a disease, but which may not result in a cure. A distinction is made between complete and partial remission. &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/remission\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">remission<\/a>) only occurs very rarely in cases of severe bone marrow failure.<\/p>\n<p><strong>Treatment is needed for:<\/strong><\/p>\n<ul>\n<li>Very severe (vSAA) and severe <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia (SAA)<\/li>\n<li>Non-severe <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia (nSAA) with a marked reduction in at least one cell line (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;cytopenia&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Reduction in the number of cells in the blood, e.g. erythro-, leuko-, granulo-, lympho- or thrombocytopenia&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/cytopenia\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">cytopenia<\/a>), which requires regular <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a>s or leads to a risk of infection or bleeding<\/li>\n<li>Transition (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;progression&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Progression of a disease&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/progression\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">progression<\/a>) from an nSAA to an SAA<\/li>\n<\/ul>\n<p>If the patient has nSAA, one can usually apply &lsquo;watchful waiting&rsquo; without intensive therapy.<\/p>\n<p>While a few decades ago there was hardly any prospect of a cure or long-term improvement, there are now promising options. Two main types of treatment are available: <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppressive&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppressive\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppressive<\/a> therapy (IST), and stem cell transplantation (SCT) or bone marrow transplantation (BMT). In addition, there are specific treatments for certain subgroups of patients. Which of these is appropriate depends on the severity of the disease, the patient&rsquo;s age and possible concomitant diseases &mdash; as well as the degree of the <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a> match (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA compatibility&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Since an unrelated donor can never be completely &amp;quot;identical&amp;quot;, we refer to HLA compatibility in this context&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla-compatibility\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">HLA compatibility<\/a>) with the potential bone marrow donor, whether related or unrelated.   <\/p>\n<p>If therapy is indicated, treatment should be started as soon as possible to avoid the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;progression&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Progression of a disease&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/progression\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">progression<\/a> of the disease and its possible complications (e.g. pronounced anaemia, infections, bleeding and coagulation disorders). Early therapy planning in cooperation with a specialised centre is therefore important. <\/p>\n<p>The course of therapy for patients with severe or very severe <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia and for patients with nSAA who require treatment is shown in the figure below:<\/p>\n<p><\/p><div id=\"attachment_3509\" style=\"width: 786px\" class=\"wp-caption alignnone\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-3509\" class=\"wp-image-3509\" src=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/2019\/07\/AA_Therapiealgorithmus.gif\" alt=\"Clearly simplified presentation of the therapy algorithm.\" width=\"776\" height=\"694\"\/><p id=\"caption-attachment-3509\" class=\"wp-caption-text\"><i>Clearly simplified presentation of the therapy algorithm. For the complete algorithm, please visit <\/i> <a href=\"https:\/\/www.onkopedia.com\/de\/onkopedia\/guidelines\/aplastische-anaemie\/@@guideline\/html\/index.html\" target=\"_blank\" rel=\"noopener noreferrer\">Onkopedia (German)<\/a><\/p><\/div>\n<p>&nbsp;<\/p>\n<h4><\/h4>\n<h4><span style=\"color: #ffcc00;\">5.2 Immunosuppressive Therapy (IST)<\/span><\/h4>\n<p>&nbsp;<\/p>\n<h5><span style=\"color: #ffcc00;\">Antithymocyte Globulin (ATG) and ciclosporin (CsA)<\/span><\/h5>\n<p>Since in <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia the body&rsquo;s own <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immune system&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Defence system; system that enables the body to fight off infections and to distinguish between its own and foreign tissues. In humans, the immune system consists of specialised proteins (antibodies), immune cells (white blood cells) and immune organs. It is responsible for our body&amp;#039;s defences(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immune-system\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immune system<\/a> turns against its own bone marrow, <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppressive&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppressive\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppressive<\/a> therapy is often indicated, especially in the case of:<\/p>\n<ul>\n<li>Patients with vSAA or SAA &gt; 40-50 years<\/li>\n<li>Patients without <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a>-identical (sibling-) donors<\/li>\n<li>Patients with nSAA at risk of severe <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;cytopenia&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Reduction in the number of cells in the blood, e.g. erythro-, leuko-, granulo-, lympho- or thrombocytopenia&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/cytopenia\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">cytopenia<\/a> in at least one cell row<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<p>Immunosuppressive therapy is usually a combination of the drugs antithymocyte globulin and <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ciclosporin&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Also known as cyclosporin A, CsA; immunosuppressive medication administered e.g. to treat aplastic anaemia and prevent GvHD disease. In high doses and with long-term administration, its main side effects are hypertension, tremor of the hands and deterioration of kidney function. Blood levels(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/ciclosporin\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">ciclosporin<\/a>. This allows the bone marrow to recover. In the course of the therapy, the blood count usually deteriorates for a short time before there is an improvement. The most common complications are fever or allergic reactions and rarely infections.   <\/p>\n<p><a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a> is an <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;antibody&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;A protein normally produced by the immune system to defend against a pathogen that has entered the body or another threat; an antibody binds specifically to certain surface structures of the intruder, and triggers a series of further immune reactions that ultimately lead to the killing and(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/antibody\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">antibody<\/a> that destroys the overactive, bone marrow-damaging T <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;lymphocytes&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Subgroup of white blood cells that play a role in the defence against diseases and foreign substances&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/lymphocytes\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">lymphocytes<\/a>. <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a> is usually given for 4-5 days as an <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;infusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Injection of a liquid into a vein&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/infusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">infusion<\/a> into a large <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;vein&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Blood vessel that leads to the heart and transports oxygen-poor blood (exception: pulmonary vein)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/vein\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>vein<\/a> via a central venous catheter (CVC). During <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a> therapy, the platelet count should be elevated to 30 G\/l or kept there by means of platelet <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a>, if necessary, as the platelet levels can drop rapidly during therapy. For an <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a> therapy, an inpatient stay of about 1-2 weeks must be expected. Side effects of <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a> therapy can be allergic reactions such as skin rash and fever. To suppress <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;acute&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Sudden onset, rapidly progressing&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/acute\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>acute<\/a> side effects of <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a>, a cortisone preparation, e.g. prednisone or prednisolone, is also administered for a short time.     <\/p>\n<p>Current data show that serum obtained from horses (equine <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a>, horse <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a>, h<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a>) is significantly more effective than rabbit <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a> (r<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a>). However, the only approved horse <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a> (h<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a>) preparation (Lymphoglobulin&reg;) was withdrawn from the market in the EU in 2007, so the medication must currently be imported from outside the EU. Due to the lack of approval in the EU, it is advisable to clarify the responsibility for costs in advance with the respective health insurance company. A new approval of equine\/horse <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a> in the EU has been applied for by the manufacturer, but not yet decided.   <\/p>\n<p>Ciclosporin, which inhibits the release of immunostimulants, is another key factor in the therapeutic response to the disease. CsA is subject to regular laboratory tests to ensure that the optimal effect is achieved by adjusting the dose, if necessary. The aim is to achieve a minimum concentration of 100-200 ng\/ml in the blood. For a stable effective level, the medication should be taken very regularly at fixed intervals of 12 hours.   <\/p>\n<p>Possible side effects of CsA therapy are infections, deterioration in kidney function, increased blood pressure, gum growth (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;gingival hyperplasia&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Overproliferation of the gums; may occur with administration of ciclosporin&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/gingival-hyperplasia\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">gingival hyperplasia<\/a>), increase in hair growth or <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;tremor&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Shaking&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/tremor\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>tremor<\/a>.<br>\nCsA is taken as a capsule or juice for at least 12 months. To ensure a good and stable response to therapy, it is important that discontinuation involves reducing the dose of CsA very slowly and gradually in order to prevent a <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;relapse&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Recurrence of disease after a period without symptoms or complaints&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/relapse\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">relapse<\/a>. However, in some patients, CsA needs to be given for a longer period or even permanently to maintain the success of the therapy.  <\/p>\n<p>By intensifying <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppressive&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppressive\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppressive<\/a> therapy, a cure (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;complete remission&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;CR (complete remission); complete regression of disease symptoms&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/complete-remission\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">complete remission<\/a>, CR) or at least a marked improvement (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;partial remission&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;PR (partial remission); decrease in disease symptoms by at least 50%&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/partial-remission\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">partial remission<\/a>, PR) can be achieved in about 50-75% of patients, with <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a> independence and a significant reduction in the risk of infection and bleeding. It takes about 2-4 months, in some patients even 6 months, until an improvement of the blood values occurs. In most cases complete normalisation of blood values cannot be achieved.  <\/p>\n<p>If there is no response, a repeat of the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppressive&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppressive\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppressive<\/a> therapy can be considered after 4-6 months.<\/p>\n<p>The risk of a <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;relapse&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Recurrence of disease after a period without symptoms or complaints&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/relapse\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">relapse<\/a> of the disease (recurrence) was about 35% earlier, when slow CsA tapering was still uncommon. The risk of <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;relapse&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Recurrence of disease after a period without symptoms or complaints&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/relapse\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">relapse<\/a> is lower with very slow CsA reduction. In the event of a <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;relapse&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Recurrence of disease after a period without symptoms or complaints&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/relapse\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">relapse<\/a>, a repeat of the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppressive&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppressive\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppressive<\/a> therapy is possible, as the chance of a renewed response is 30-60%.  <\/p>\n<p>In addition to specific therapy, every patient should receive <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;supportive&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;help other than with a disease-specific drug&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/supportive\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">supportive<\/a> therapy, see <span style=\"color: #000080;\"><a style=\"color: #000080;\" href=\"#suppther\">usupportive therapy<\/a><\/span> .<\/p>\n<h5><span style=\"color: #ffcc00;\">Alemtuzumab<\/span><\/h5>\n<p>There are also other drugs that work through the same mechanism of <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppression&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppression\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppression<\/a>. These include alemtuzumab, an <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;antibody&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;A protein normally produced by the immune system to defend against a pathogen that has entered the body or another threat; an antibody binds specifically to certain surface structures of the intruder, and triggers a series of further immune reactions that ultimately lead to the killing and(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/antibody\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">antibody<\/a> that works against T <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;lymphocytes&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Subgroup of white blood cells that play a role in the defence against diseases and foreign substances&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/lymphocytes\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">lymphocytes<\/a>. This drug is used to treat <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;chronic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Over a long period of time and persistent&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/chronic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">chronic<\/a> lymphocytic leukaemia (CLL) or multiple sclerosis (MS), but it has also shown good response rates in trials of <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia, especially in older patients. One advantage of this drug is that it is only injected under the skin, so no hospital stay is necessary. If the patient previously had an infection with the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;cytomegalovirus&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;CMV; virus of the herpes group, which can cause serious complications in organ transplanted and immunocompromised patients. The pathogen is widespread in the population. The virus remains in the body for life after initial infection and can be reactivated if the immune system is weakened.   &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/cytomegalovirus\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">cytomegalovirus<\/a> (CMV), this blood value should be checked regularly, as this viral infection can reoccur under therapy.    <\/p>\n<p>Patients who did not respond to other therapies showed response rates of 37-48% when treated with alemtuzumab.<\/p>\n<h4><span style=\"color: #ffcc00;\">5.3 Allogeneic transplantation<\/span><\/h4>\n<p>&nbsp;<\/p>\n<p>In patients up to the age of approximately 50 years with severe or very severe <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia (SAA or vSAA) and availability of a sibling donor who is fully compatible with the patient with respect to compatibility markers (<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a>) (<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a>-identical), the preferred treatment (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;first-line therapy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;First-choice therapy, may be described in a guideline as the most suitable treatment&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/first-line-therapy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">first-line therapy<\/a>) is an allogeneic transplantation.<\/p>\n<p>Patients younger than 18 years of age can also receive stem cells from an unrelated <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a>-identical donor (foreign donor) if they do not have an <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a>-identical family donor. It is important that &lsquo;fine mapping&rsquo; is performed and that donor and recipient are completely identical in this regard.<br>\nIn recent years, the complication rate for <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a>-identical foreign donor transplantation has been significantly reduced, so that it is increasingly used &ndash; especially in patients up to 40 years of age who do not respond to <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppressive&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppressive\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppressive<\/a> treatment.<\/p>\n<p>The aim of allogeneic transplantation is to replace the patient&rsquo;s non-functional bone marrow with healthy stem cells from a donor. To achieve this, the patient&rsquo;s bone marrow is first destroyed by various measures (chemotherapy, <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;antibody&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;A protein normally produced by the immune system to defend against a pathogen that has entered the body or another threat; an antibody binds specifically to certain surface structures of the intruder, and triggers a series of further immune reactions that ultimately lead to the killing and(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/antibody\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">antibody<\/a> therapy, radiation). This &lsquo;<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;conditioning&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Preparation of a patient for stem cell transplantation in the form of high-dose chemotherapy (high-dose therapy) and, if necessary, whole-body radiation. The recipient&amp;#039;s immune system must be suppressed to such an extent that the donor&amp;#039;s transplant can grow without the risk of rejection. &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/conditioning\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">conditioning<\/a>&rsquo; is carried out in the days immediately before the transplantation.<br>\nIn parallel, new, healthy stem cells are collected from a healthy, related or unrelated volunteer.<br>\nStem cells can be obtained directly from the bone marrow with <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;bone marrow biopsy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Taking a sample from the bone marrow (usually from the iliac crest) with a tissue punch. This method enables more precise examinations to be carried out than with bone marrow aspiration, since the bone punch cylinder is also obtained. &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/bone-marrow-biopsy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">bone marrow biopsy<\/a> under anaesthesia. The punctures in the iliac crest for bone marrow collection may cause bruising and pain that may last for several days. In addition, there is the general anaesthetic risk.  <\/p>\n<p>Alternatively, the donor is injected with a drug that stimulates <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;granulocyte&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Subgroup of white blood cells; they destroy invading bacteria that can cause diseases &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/granulocyte\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">granulocyte<\/a> development (<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;G-CSF&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;G-CSF; genetically engineered drug that promotes the development of granulocytes and leads to washout of stem cells from the bone marrow into the blood. It is tolerated quite well, but may cause temporary fever and limb pain in a small proportion of patients. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/g-csf\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>G-CSF<\/a>) over several days. The increased number of blood stem cells produced in this way migrate from the bone marrow into the blood. These &lsquo;<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;peripheral&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Area outside a centre or origin; peripheral blood is blood in the peripheral circulation&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/peripheral\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">peripheral<\/a> blood stem cells&rsquo; (<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;PBSC&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;peripheral blood stem cells&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/pbsc\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>PBSC<\/a>) are then removed with a special device (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;apheresis&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Targeted removal of certain components from the blood using a special apparatus&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/apheresis\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">apheresis<\/a>), as in a blood plasma donation. The procedure can lead to flu-like symptoms and pain.   <\/p>\n<p>If the stem cells are obtained directly from the bone marrow, this is called a bone marrow transplantation (BMT). If the stem cells are obtained by <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;apheresis&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Targeted removal of certain components from the blood using a special apparatus&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/apheresis\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">apheresis<\/a>, this is called a stem cell transplantation (SCT).<br>\nStudies suggest that treatment of <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia with stem cells from <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;peripheral&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Area outside a centre or origin; peripheral blood is blood in the peripheral circulation&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/peripheral\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">peripheral<\/a> blood may be associated with increased complications such as <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;acute&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Sudden onset, rapidly progressing&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/acute\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>acute<\/a> or <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;chronic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Over a long period of time and persistent&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/chronic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">chronic<\/a> rejection. If possible, stem cells obtained directly from the bone marrow should therefore be used. <\/p>\n<p>Regardless of the method of obtaining the stem cells, they are purified and examined for infectious agents. The patient then receives the healthy stem cells. The transplantation itself is like a blood <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a>. If everything goes well, the donor stem cells &ldquo;grow&rdquo; and lead to normal bone marrow function and blood development. An inpatient stay of at least four weeks is required for a transplant.    <\/p>\n<p>During the transplantation, the patient receives <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;prophylactic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Prevention or precaution&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/prophylactic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">prophylactic<\/a> medication to prevent infections caused by bacteria and fungi. In addition, a cortisone preparation, e.g. prednisolone, and <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ciclosporin&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Also known as cyclosporin A, CsA; immunosuppressive medication administered e.g. to treat aplastic anaemia and prevent GvHD disease. In high doses and with long-term administration, its main side effects are hypertension, tremor of the hands and deterioration of kidney function. Blood levels(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/ciclosporin\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">ciclosporin<\/a> (CsA) are administered to influence the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immune system&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Defence system; system that enables the body to fight off infections and to distinguish between its own and foreign tissues. In humans, the immune system consists of specialised proteins (antibodies), immune cells (white blood cells) and immune organs. It is responsible for our body&amp;#039;s defences(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immune-system\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immune system<\/a> for several months. <\/p>\n<p><strong>Potential complications due to the transplantation are:<\/strong><\/p>\n<ul>\n<li>Toxic side effects during <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;conditioning&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Preparation of a patient for stem cell transplantation in the form of high-dose chemotherapy (high-dose therapy) and, if necessary, whole-body radiation. The recipient&amp;#039;s immune system must be suppressed to such an extent that the donor&amp;#039;s transplant can grow without the risk of rejection. &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/conditioning\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">conditioning<\/a> therapy<\/li>\n<li>Infections<\/li>\n<li><span style=\"color: #000080;\"><a style=\"color: #000080;\" href=\"#gvhd\">Graft versus Host-Disease (GvHD)<\/a><\/span><\/li>\n<li>Transplant rejection<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<p><strong><a id=\"gvhd\"><\/a>Graft versus Host-Disease (<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;GvHD&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;GvHD; graft versus recipient response. The donor&amp;#039;s immune system transplanted with a foreign donation (e.g. stem cells) can recognise the recipient&amp;#039;s body cells as foreign and attack them. A distinction is made between acute GvHD (grades 1-4) and chronic GvHD (limited and extensive form). The(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/gvhd\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>GvHD<\/a>):<\/strong> Here the donated <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immune system&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Defence system; system that enables the body to fight off infections and to distinguish between its own and foreign tissues. In humans, the immune system consists of specialised proteins (antibodies), immune cells (white blood cells) and immune organs. It is responsible for our body&amp;#039;s defences(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immune-system\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immune system<\/a> reacts against the body&rsquo;s own cells. This can be short term (<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;acute&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Sudden onset, rapidly progressing&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/acute\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>acute<\/a>) or long lasting (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;chronic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Over a long period of time and persistent&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/chronic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">chronic<\/a>), so that under certain circumstances permanent suppression of the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immune system&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Defence system; system that enables the body to fight off infections and to distinguish between its own and foreign tissues. In humans, the immune system consists of specialised proteins (antibodies), immune cells (white blood cells) and immune organs. It is responsible for our body&amp;#039;s defences(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immune-system\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immune system<\/a> (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppressive&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppressive\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppressive<\/a> therapy) may be necessary. <\/p>\n<p>&nbsp;<\/p>\n<h4><\/h4>\n<h4><span style=\"color: #ffcc00;\">5.4 Further treatment options<\/span><\/h4>\n<p>&nbsp;<\/p>\n<h5><span style=\"color: #ffcc00;\">Danazol<\/span><\/h5>\n<p>Some patients with <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia have a rare congenital disorder in which the ends of the chromosomes (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;telomere&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;End of chromosomes that shorten with each cell division&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/telomere\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">telomere<\/a>s) are shortened, which is called <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;telomeropathy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Disease in which telomere shortening is exacerbated&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/telomeropathy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">telomeropathy<\/a>. The shortening of the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;telomere&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;End of chromosomes that shorten with each cell division&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/telomere\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">telomere<\/a>s results in disrupted cell division and thus to reduced development of blood cells in the bone marrow. <\/p>\n<p>Danazol, a synthetic variant of the male sex hormone testosterone, may cause the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;telomere&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;End of chromosomes that shorten with each cell division&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/telomere\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">telomere<\/a>s to lengthen, which can lead to an improvement in symptoms and even normal blood development.<\/p>\n<h5><span style=\"color: #ffcc00;\">Eltrombopag (Revolade&reg;)<\/span><\/h5>\n<p>Eltrombopag has been approved for adult patients with acquired severe <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia (SAA) since 2015 if they:<\/p>\n<ul>\n<li>have not responded to previous <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppressive&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppressive\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppressive<\/a> therapy, or<\/li>\n<li>have received intensive pre-treatment and<\/li>\n<li>are not suitable for bone marrow or stem cell transplantation.<\/li>\n<\/ul>\n<p>Eltrombopag acts to control the development of blood stem cells and platelets. The medication activates thrombopoietin, which controls the development of platelets and blood cells (haematopoiesis). The initial dose is 50 mg\/day (maximum dose 150 mg\/day). In the authorisation trial, it was shown that the use of eltrombopag led to an improvement of platelet, <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;erythrocyte&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Red blood cell; erythrocytes make up the majority of cellular blood components. They are formed in the bone marrow, contain haemoglobin (the red pigment in blood ) and have a life expectancy of about 120 days in healthy people. The haemoglobin content of the blood is an important measurement(...)&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/erythrocyte\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">erythrocyte<\/a> and <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;neutrophil&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Subgroup of granulocytes with an important role in defending against bacterial and fungal infections&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/neutrophil\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">neutrophil<\/a> levels in some patients.    Eltrombopag resulted in an improvement or normalisation of bone marrow cellularity. Patients who had previously required regular <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a>s had an increased number of days until the next <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a> or became <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a>-independent. Due to the very good response rates in clinical trials, eltrombopag has already been approved in the USA in combination with h<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a> and CsA for first-line treatment of <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia. An application for approval in the EU has also been filed.   <\/p>\n<h5><span style=\"color: #ffcc00;\">Other<\/span><\/h5>\n<p>Therapies without proven efficacy, e.g. steroid <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;monotherapy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Medication with only one active substance&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/monotherapy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">monotherapy<\/a> or <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;monotherapy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Medication with only one active substance&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/monotherapy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">monotherapy<\/a> with haematopoietic growth factors, should be avoided, as they only mean loss of time and can significantly worsen the initial situation of the patient with regard to one of the proven therapeutic options.<\/p>\n<p>&nbsp;<\/p>\n<h4><a id=\"suppther\"><\/a><span style=\"color: #ffcc00;\">5.5 Supportive Therapy<\/span><\/h4>\n<p>&nbsp;<\/p>\n<h5><a id=\"tx\"><\/a><br>\n<span style=\"color: #ffcc00;\">Blood Transfusions<\/span><\/h5>\n<p>Transfusions are necessary for many patients to ensure adequate physical fitness and quality of life and to avoid bleeding complications. They can temporarily replace the missing blood cells in case of corresponding symptoms (anaemia, bleeding). Not all of the blood is <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfuse&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfuse\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfuse<\/a>d, but only the type of cell that is needed (red blood cells or platelets).  <\/p>\n<p><strong>To do this, the blood is examined after a blood donation to rule out transmissible infections. The white blood cells are then removed, and finally the various blood components are separated and concentrated. Family members are not allowed to donate blood, as these so-called directed donations pose particular risks. <\/strong><\/p>\n<p>In order to ensure satisfactory tolerance, the preparation used is not only selected according to blood group (A, B, AB, O) and rhesus factor, but each individual preparation is tested individually for each patient. For this purpose, in a compatibility test or &lsquo;cross-matching&rsquo;, the patient&rsquo;s blood is mixed with blood from the packed red blood cells (PRBCs) and examined. Especially if antibodies are present, it can take longer to find suitable PRBCs. If a patient is <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;cytomegalovirus&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;CMV; virus of the herpes group, which can cause serious complications in organ transplanted and immunocompromised patients. The pathogen is widespread in the population. The virus remains in the body for life after initial infection and can be reactivated if the immune system is weakened.   &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/cytomegalovirus\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">cytomegalovirus<\/a> (CMV) negative and there is a possibility of a later bone marrow or stem cell transplantation, CMV-negative concentrates should be given.<br>\nIn general, packed red blood cells should be used with caution, as they can lead to the body being overloaded with iron. <strong>Indications for a <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a> are:<\/strong><\/p>\n<ul>\n<li>Pronounced reduction in stamina associated with <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;fatigue&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Severe tiredness, exhaustion or increased need for rest; a symptom that often accompanies various chronic diseasesitet&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/fatigue\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">fatigue<\/a> or in the context of shortness of breath, e.g. under physical exertion, and depending on the respective concomitant diseases, e.g. heart failure<\/li>\n<li>A very low <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;haemoglobin&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Hb; red blood cell pigment consisting of haem (an iron-containing component) and globin (a protein). Binds, transports and releases oxygen and carbon dioxide. Reference values: women 12-16 g\/dl or 7.5-9.9 mmol\/l, men 14-18 g\/dl or 8.7-11.2 mmol\/l &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/haemoglobin\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">haemoglobin<\/a> level (&lt; 7 g\/dl)<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<p>Each unit of packed red blood cells absorbs more than 100 times the amount of iron than is taken in daily with food. As the human body cannot actively excrete iron, it is deposited in various organs, especially the liver, heart, kidney and bone marrow (see Table 2) and can damage them. Patients with <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia or myelodysplastic syndrome (MDS) are particularly at risk because the impaired bone marrow function already decreases the development of new red blood cells and the iron cannot be used completely for the development of new ones.  <\/p>\n<p><\/p><div id=\"attachment_3511\" style=\"width: 507px\" class=\"wp-caption alignnone\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-3511\" class=\"wp-image-3511 size-full\" src=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/2019\/07\/AA_Fe_Ueberl_web.jpg\" alt=\"Diagnosis and therapy of AA: Areas of potential iron overload\" width=\"497\" height=\"242\"\/><p id=\"caption-attachment-3511\" class=\"wp-caption-text\"><i>Complications of iron overload<\/i><\/p><\/div>\n<p>&nbsp;<\/p>\n<p>As a rule, in the first few months after diagnosis, no storage iron (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ferritin&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Protein that stores iron; if ECs are given frequently, the ferritin level should be monitored in case chelation therapy becomes necessary.&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/ferritin\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">ferritin<\/a>) or liver iron levels are reached which require immediate therapy to bind and excrete the excess iron (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;chelation therapy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Therapy for removal of excess iron, which was stored e.g. by increased transfusions of erythrocyte concentrates&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/chelation-therapy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">chelation therapy<\/a>). It is therefore advisable to wait at least 6 months after initiating <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppression&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppression\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppression<\/a>. If there is a continuing need for regular <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a>s and serum <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ferritin&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Protein that stores iron; if ECs are given frequently, the ferritin level should be monitored in case chelation therapy becomes necessary.&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/ferritin\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">ferritin<\/a> levels are above 1,000 &micro;g\/l, <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;chelation therapy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Therapy for removal of excess iron, which was stored e.g. by increased transfusions of erythrocyte concentrates&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/chelation-therapy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">chelation therapy<\/a> is indicated. This is especially true for transplant candidates, as iron overload is associated with higher transplant-related <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;mortality&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Death rate&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/mortality\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">mortality<\/a> and <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;morbidity&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Frequency of illness; complaints and complications caused by an illness or during therapy&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/morbidity\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">morbidity<\/a>.   <\/p>\n<p>The drugs used today to treat iron overload are generally well tolerated. The major side effects include nausea, diarrhoea and <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;renal&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Involving the kidney&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/renal\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>renal<\/a> dysfunction, but these disappear after discontinuation. <\/p>\n<p>If the serum <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ferritin&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Protein that stores iron; if ECs are given frequently, the ferritin level should be monitored in case chelation therapy becomes necessary.&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/ferritin\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">ferritin<\/a> is permanently below 500 &micro;g\/l and there is <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a>-related iron overload, interrupting treatment may be considered depending on individual <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a> needs. However, this should always be done in consultation with the treating doctor. <\/p>\n<p><strong>Further information on this issue can be found in the <a href=\"https:\/\/www.leukaemie-hilfe.de\/fileadmin\/user_upload\/dlh-broschueren\/DLHS_21_Eisenueberladung.pdf\" target=\"_blank\" rel=\"noopener noreferrer\">Brochure &bdquo;Transfusion-Induced Iron Overload&rdquo;<\/a> (German) by the Deutsche Leuk&auml;mie- &amp; Lymphom-Hilfe.<\/strong><\/p>\n<h5><span style=\"color: #ffcc00;\">Infections<\/span><\/h5>\n<p>In the case of febrile infections, a doctor should be consulted as soon as possible to make a diagnosis and initiate therapy.<\/p>\n<p>In certain cases, the preventive use of antibiotics aghttps:\/\/www.leukaemie-hilfe.de\/fileadmin\/user_upload\/DLH_Broschuere_Eisenueberladung_2024_lowres.pdf ainst bacteria and of antimycotics (&lsquo;against fungi&rsquo;) can be useful.<strong>In addition, if the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;granulocyte&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Subgroup of white blood cells; they destroy invading bacteria that can cause diseases &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/granulocyte\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">granulocyte<\/a>\/<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;neutrophil&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Subgroup of granulocytes with an important role in defending against bacterial and fungal infections&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/neutrophil\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">neutrophil<\/a> count is low (&lt; : (&lt; 0.5\/nl), various behavioural measures should be observed<\/strong><\/p>\n<ul>\n<li>Avoid contact with people who have infections<\/li>\n<li>Avoid close physical contact with animals<\/li>\n<li>Avoid large crowds, especially in the winter months<\/li>\n<li>Carry out usual hygiene measures, e.g. washing hands, oral hygiene, and ensure freshness and cleaning of raw food<\/li>\n<li>Avoid close contact with fungal spores, especially gardening, do not clean organic waste bins or turn compost<\/li>\n<\/ul>\n<p>\nIn very rare cases, e.g. severe infections, the use of the haematopoietic growth factors <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;G-CSF&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;G-CSF; genetically engineered drug that promotes the development of granulocytes and leads to washout of stem cells from the bone marrow into the blood. It is tolerated quite well, but may cause temporary fever and limb pain in a small proportion of patients. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/g-csf\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>G-CSF<\/a> or GM-CSF to stimulate the body&rsquo;s immune defence or the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a> of white blood cell concentrates (<a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;granulocyte&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Subgroup of white blood cells; they destroy invading bacteria that can cause diseases &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/granulocyte\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">granulocyte<\/a> concentrates) may be considered.<\/p>\n<p><strong>You can find more detailed information in the <a href=\"https:\/\/www.leukaemie-hilfe.de\/fileadmin\/user_upload\/dlh-broschueren\/2025_Hygiene_Immunsuppression_Patientenbroschuere_DLH_VAH_web_final.pdf\" target=\"_blank\" rel=\"noopener noreferrer\">Brochure &bdquo;Infections? No thanks!&rdquo;<\/a> (German) by M. Exner, A. Simon and Deutsche Leuk&auml;mie- &amp; Lymphom-Hilfe.<\/strong><\/p>\n<h5><span style=\"color: #ffcc00;\">Bleeding<\/span><\/h5>\n<p>In case of bleeding, platelet concentrates (PCs) can be transferred to avoid complications. As bleeding with low platelet counts can be life-threatening <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;acute&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Sudden onset, rapidly progressing&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/acute\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>acute<\/a> emergencies, immediate action must be taken in these cases. If the platelet count is very low, platelet concentrates can also be given as a preventive measure. The life span of platelets is only a few days. If there is very little or no production of platelets in the bone marrow, the administration of platelet concentrates may therefore be necessary several times a week.    <\/p>\n<p>Platelets carry tissue characteristics (<a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a> markers) that are different for each person. Some patients have antibodies against these <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a> markers. This can happen spontaneously, during illness or after pregnancy. If such <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a> antibodies are present, the <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfuse&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfuse\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfuse<\/a>d blood platelets are immediately destroyed and there is not a sufficient increase in platelets after a <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;transfusion&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Transfer of blood or blood components&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/transfusion\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">transfusion<\/a> of a platelet concentrate. For these patients, special <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a>-compatible platelet concentrates must be prepared from donors with matching <a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a> characteristics.    <\/p>\n<p>The use of platelet aggregation inhibitors such as acetylsalicylic acid (ASS) should be considered very carefully, particularly with very low platelet counts.<\/p>\n<p>In women who have severe problems with menstrual bleeding, blood loss due to platelet deficiency can also be temporarily discontinued with hormone therapy, e.g. continuous administration of contraceptive pills or 3-monthly injections.<\/p>\n<h5><span style=\"color: #ffcc00;\">activities<\/span><\/h5>\n<p>BIn patients with <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia, physical activity and exercise are recommended, depending on blood levels and the patient&rsquo;s state of health. However, care should be taken to ensure that no excessive demands are made. It is therefore advisable to monitor the pulse rate during exercise. This is particularly important in anaemia, because if the red blood cell count is low, the body often tries to compensate for this deficiency by increasing the heart rate, which can lead to excessive strain on the heart. In the case of <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;thrombocytopenia&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Platelet deficiency&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/thrombocytopenia\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">thrombocytopenia<\/a>, sports with a risk of injury (e.g. martial arts or rock climbing), should be avoided at all costs.    <\/p>\n<h5><span style=\"color: #ffcc00;\">Rehabilitation<\/span><\/h5>\n<p>If one&rsquo;s participation in &ldquo;normal&rdquo; life can no longer be carried out as usual due to <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia, rehabilitation measures, outpatient physiotherapy or psychological or psychotherapeutic care may be appropriate. These measures should be individually tailored to the patient.<br>\nIf intensive therapy measures are planned, it makes sense to carry out rehabilitation measures only after these therapies. Physiotherapy or a psychological or psychotherapeutic care is also helpful to accompany the therapy. <\/p>\n<p>If a child suffering from <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;aplastic&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Condition of the non-functional bone marrow that cannot develop blood cells&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/aplastic\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">aplastic<\/a> anaemia is reintegrated after treatment with <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;immunosuppressive&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Suppression of the immune system&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/immunosuppressive\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">immunosuppressive<\/a> therapy or transplantation, a family-oriented measure in a paediatric-oncological aftercare facility can be useful due to the high psychosocial burden on the families.<\/p>\n<p><\/p><\/ul><ul style=\"margin-top:0;margin-bottom:0\"><style type=\"text\/css\">a.bg-showmore-plg-link:hover,a.bg-showmore-plg-link:active,a.bg-showmore-plg-link:focus{color:#0071bb;}<\/style>\n<input type=\"hidden\" bg_collapse_expand_pro=\"69e3e86a1a4d57096799986\" value=\"69e3e86a1a4d57096799986\"><input type=\"hidden\" id=\"bg-show-more-text-69e3e86a1a4d57096799986\" value=\"      6 Prognosis\"><input type=\"hidden\" id=\"bg-show-less-text-69e3e86a1a4d57096799986\" value=\"      6 Prognosis\"><input type=\"hidden\" id=\"bg-icon-bg-color-69e3e86a1a4d57096799986\" value=\"\"><a id=\"bg-showmore-action-69e3e86a1a4d57096799986\" class=\"bg-showmore-plg-link bg-arrow  \" style=\"color:#ffcc00;;font-weight:bold; font-size:24px; line-height: 150%\" href=\"#\">      6 Prognosis<\/a><\/ul><ul class=\"bg-showmore-no-padding-no-margin bg-showmore-closed\" id=\"bg-showmore-pro-hidden-69e3e86a1a4d57096799986\">\n<p>The higher the <em><a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;granulocyte&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Subgroup of white blood cells; they destroy invading bacteria that can cause diseases &lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/granulocyte\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">granulocyte<\/a><\/em>count and the lower the age of the patient at the time of diagnosis, the better the prognosis.<\/p>\n<p>The data on survival after different therapies listed below are statistical data. This means that they cannot be automatically transferred to the individual patient. This list is only intended to give an overview of how possibilities and survival have improved in recent years. There are often subgroups that are not included here. For all specific forms of therapy, the results are significantly better for patients under 20 years of age than for patients over 20. The same applies to patients under 40 years of age compared to patients over 40 years of age. For stem cell transplants, the results are considerably better if the donor provides stem cells directly from the bone marrow and not <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;peripheral&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Area outside a centre or origin; peripheral blood is blood in the peripheral circulation&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/peripheral\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">peripheral<\/a> <em><a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;peripheral&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Area outside a centre or origin; peripheral blood is blood in the peripheral circulation&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/peripheral\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">peripheral<\/a><\/em> stem cells obtained from the blood.      <\/p>\n<p><strong>According to published data, overall survival in SAA\/vSAA is after 3-6 years and separately for the different specific therapies:<\/strong><\/p>\n<ul>\n<li>After allogeneic SCT from a <em><a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a>-identical<\/em> family donor: 75-90%<\/li>\n<li>After allogeneic SCT of <em><a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;HLA&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;human leukocyte antigen; protein structure on the surface of most body cells. They help the immune system to differentiate between &amp;quot;self&amp;quot; and &amp;quot;foreign&amp;quot; material. &lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/hla\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>HLA<\/a>-identical<\/em>unrelated donors: 65-73 %<\/li>\n<li>After <em><a class=\"glossaryLink\"  aria-describedby=\"tt\"  data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;ATG&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;ATG; immunosuppressive mixture of antibodies obtained from rabbits or horses. It reduces the number of lymphocytes (lymphocytopenia), mainly by suppressing circulating T-lymphocytes. It is used for instance to treat aplastic anaemia and for the prophylaxis and therapy of GvHD in the context of(...)&lt;\/div&gt;\"  href=\"https:\/\/aa-pnh.org\/en\/glossar\/atg\/\"  data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]'  tabindex='0' role='link'>ATG<\/a><\/em>\/<em>CsA<\/em> therapy: 76-96 %<\/li>\n<\/ul>\n<p><\/p><\/ul><ul style=\"margin-top:0;margin-bottom:0\"><style type=\"text\/css\">a.bg-showmore-plg-link:hover,a.bg-showmore-plg-link:active,a.bg-showmore-plg-link:focus{color:#0071bb;}<\/style>\n<input type=\"hidden\" bg_collapse_expand_pro=\"69e3e86a1a5729005310528\" value=\"69e3e86a1a5729005310528\"><input type=\"hidden\" id=\"bg-show-more-text-69e3e86a1a5729005310528\" value=\"      7 Registry\"><input type=\"hidden\" id=\"bg-show-less-text-69e3e86a1a5729005310528\" value=\"      7 Registry\"><input type=\"hidden\" id=\"bg-icon-bg-color-69e3e86a1a5729005310528\" value=\"\"><a id=\"bg-showmore-action-69e3e86a1a5729005310528\" class=\"bg-showmore-plg-link bg-arrow  \" style=\"color:#ffcc00;;font-weight:bold; font-size:24px; line-height: 150%\" href=\"#\">      7 Registry<\/a><\/ul><ul class=\"bg-showmore-no-padding-no-margin bg-showmore-closed\" id=\"bg-showmore-pro-hidden-69e3e86a1a5729005310528\">\n<p><strong>Patients with evidence of a <a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;PNH clone&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;All blood cells affected by the mutation typical of PNH; the size of the PNH clone is a measure of the severity of the disease&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/pnh-clone\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">PNH clone<\/a><\/strong> can be entered in the international PNH Registry at the University Hospital in Essen in order to extend our knowledge about this group of AA patients.<\/p>\n<p><strong>If you are interested or have any questions, please email Prof Dr Alexander R&ouml;th<a href=\"mailto:alexander.roeth@uk-essen.de\">alexander.roeth(at)uk-essen.de<\/a>.<\/strong><\/p>\n<p><strong>Patients with a <em><a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;telomeropathy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Disease in which telomere shortening is exacerbated&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/telomeropathy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">telomeropathy<\/a><\/em><\/strong> can be included in the<em><a class=\"glossaryLink\" aria-describedby=\"tt\" data-cmtooltip=\"&lt;div class=glossaryItemTitle&gt;telomeropathy&lt;\/div&gt;&lt;div class=glossaryItemBody&gt;Disease in which telomere shortening is exacerbated&lt;\/div&gt;\" href=\"https:\/\/aa-pnh.org\/en\/glossar\/telomeropathy\/\" data-gt-translate-attributes='[{\"attribute\":\"data-cmtooltip\", \"format\":\"html\"}]' tabindex=\"0\" role=\"link\">telomeropathy<\/a><\/em>register at the Aachen University Hospital in order to gain further insights into this subgroup of AA patients.<\/p>\n<p><strong>If you are interested or have any questions, please email Prof Dr Tim H. Br&uuml;mmendorf<a href=\"mailto:tbruemmendorf@ukaachen.de\">tbruemmendorf(at)ukaachen.de<\/a>.<\/strong><\/p>\n<p><\/p><\/ul><ul style=\"margin-top:0;margin-bottom:0\"><style type=\"text\/css\">a.bg-showmore-plg-link:hover,a.bg-showmore-plg-link:active,a.bg-showmore-plg-link:focus{color:#0071bb;}<\/style><br>\n&nbsp;<br>\n&nbsp;\n<\/ul>","protected":false},"excerpt":{"rendered":"<p>APLASTIC ANAEMIA (AA) &nbsp; &nbsp; &nbsp;<\/p>\n","protected":false},"author":7,"featured_media":0,"parent":3689,"menu_order":23,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-3695","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/aa-pnh.org\/en\/wp-json\/wp\/v2\/pages\/3695","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/aa-pnh.org\/en\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/aa-pnh.org\/en\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/aa-pnh.org\/en\/wp-json\/wp\/v2\/users\/7"}],"replies":[{"embeddable":true,"href":"https:\/\/aa-pnh.org\/en\/wp-json\/wp\/v2\/comments?post=3695"}],"version-history":[{"count":2,"href":"https:\/\/aa-pnh.org\/en\/wp-json\/wp\/v2\/pages\/3695\/revisions"}],"predecessor-version":[{"id":9230,"href":"https:\/\/aa-pnh.org\/en\/wp-json\/wp\/v2\/pages\/3695\/revisions\/9230"}],"up":[{"embeddable":true,"href":"https:\/\/aa-pnh.org\/en\/wp-json\/wp\/v2\/pages\/3689"}],"wp:attachment":[{"href":"https:\/\/aa-pnh.org\/en\/wp-json\/wp\/v2\/media?parent=3695"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}