{"id":8975,"date":"2025-10-05T13:49:46","date_gmt":"2025-10-05T11:49:46","guid":{"rendered":"https:\/\/aa-pnh.org\/?page_id=8975"},"modified":"2026-04-04T13:55:04","modified_gmt":"2026-04-04T11:55:04","slug":"pnh","status":"publish","type":"page","link":"https:\/\/aa-pnh.org\/vi\/diseases\/pnh\/","title":{"rendered":"PNH"},"content":{"rendered":"<h1><span style=\"color: #f9a801\">B\u1ec7nh hemoglobin ni\u1ec7u k\u1ecbch ph\u00e1t ban \u0111\u00eam (PNH)<\/span><\/h1>\n<p><!-- N\u1ed9i dung --><\/p>\n<h4><a href=\"#pnhwasist\">1 PNH l\u00e0 g\u00ec<\/a><\/h4>\n<h4><a href=\"#pnhsymptome\">2 Tri\u1ec7u ch\u1ee9ng<\/a><\/h4>\n<h4><a href=\"#pnhdiagnostik\">3 Ch\u1ea9n \u0111o\u00e1n<\/a><\/h4>\n<h4><a href=\"#pnhverlauf\">4 Di\u1ec5n bi\u1ebfn l\u00e2m s\u00e0ng<\/a><\/h4>\n<h4><a href=\"#pnhtherapie\">5 \u0110i\u1ec1u tr\u1ecb<\/a><\/h4>\n<h4><a href=\"#pnhprognose\">6 Ti\u00ean l\u01b0\u1ee3ng<\/a><\/h4>\n<h4><a href=\"#pnhkinderwunsch\">7 Mong mu\u1ed1n c\u00f3 con\/mang thai<\/a><\/h4>\n<h4><a href=\"#pnhregister\">8 \u0110\u0103ng k\u00fd<\/a><\/h4>\n<p>&nbsp;<\/p>\n<h2><a name=\"pnhwasist\"><\/a><span style=\"color: #f9a801\">1 PNH l\u00e0 g\u00ec<\/span><\/h2>\n<p>&nbsp;<\/p>\n<h3><span style=\"color: #f9a801\">1.1 T\u1ed5ng quan<\/span><\/h3>\n<p>Gi\u1ed1ng nh\u01b0 thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n, hemoglobin ni\u1ec7u k\u1ecbch ph\u00e1t ban \u0111\u00eam (PNH) kh\u00f4ng ph\u1ea3i l\u00e0 b\u1ec7nh \u00e1c t\u00ednh, nh\u01b0ng l\u00e0 b\u1ec7nh r\u1ea5t hi\u1ebfm g\u1eb7p v\u00e0 c\u00f3 th\u1ec3 \u0111e d\u1ecda t\u00ednh m\u1ea1ng. B\u1ec7nh n\u00e0y do m\u1ed9t khi\u1ebfm khuy\u1ebft di truy\u1ec1n m\u1eafc ph\u1ea3i c\u1ee7a c\u00e1c t\u1ebf b\u00e0o g\u1ed1c t\u1ea1o m\u00e1u trong t\u1ee7y x\u01b0\u01a1ng, kh\u00f4ng ph\u1ea3i l\u00e0 b\u1ec7nh di truy\u1ec1n.<\/p>\n<h3><span style=\"color: #f9a801\">1.2 T\u1ef7 l\u1ec7 m\u1eafc b\u1ec7nh (d\u1ecbch t\u1ec5 h\u1ecdc)<\/span><\/h3>\n<p>T\u1ef7 l\u1ec7 m\u1eafc b\u1ec7nh (t\u1ef7 l\u1ec7 m\u1eafc) l\u00e0 1-2 ca m\u1edbi tr\u00ean 1 tri\u1ec7u ng\u01b0\u1eddi m\u1ed7i n\u0103m. Tuy nhi\u00ean, do c\u00e1c tri\u1ec7u ch\u1ee9ng r\u1ea5t \u0111a d\u1ea1ng, c\u00f3 th\u1ec3 gi\u1ea3 \u0111\u1ecbnh r\u1eb1ng PNH hi\u1ebfm khi \u0111\u01b0\u1ee3c ch\u1ea9n \u0111o\u00e1n ho\u1eb7c ch\u1ec9 \u0111\u01b0\u1ee3c ch\u1ea9n \u0111o\u00e1n mu\u1ed9n, v\u00ec b\u1ec7nh n\u00e0y th\u01b0\u1eddng kh\u00f4ng \u0111\u01b0\u1ee3c ph\u00e1t hi\u1ec7n ngay l\u1eadp t\u1ee9c. B\u1ec7nh th\u01b0\u1eddng \u0111\u01b0\u1ee3c ch\u1ea9n \u0111o\u00e1n nh\u1ea5t \u1edf \u0111\u1ed9 tu\u1ed5i t\u1eeb 25 \u0111\u1ebfn 45, v\u1edbi t\u1ef7 l\u1ec7 m\u1eafc b\u1ec7nh \u1edf nam v\u00e0 n\u1eef g\u1ea7n nh\u01b0 b\u1eb1ng nhau. Kh\u00f4ng c\u00f3 s\u1ef1 t\u1eadp trung gia \u0111\u00ecnh.<\/p>\n<h3><span style=\"color: #f9a801\">1.3 Ngu\u1ed3n g\u1ed1c (b\u1ec7nh sinh)<\/span><\/h3>\n<p>PNH x\u1ea3y ra do m\u1ed9t s\u1ef1 thay \u0111\u1ed5i di truy\u1ec1n (\u0111\u1ed9t bi\u1ebfn) c\u1ee7a c\u00e1c t\u1ebf b\u00e0o g\u1ed1c t\u1ea1o m\u00e1u trong t\u1ee7y x\u01b0\u01a1ng. S\u1ef1 thay \u0111\u1ed5i n\u00e0y kh\u00f4ng c\u00f3 t\u1eeb khi sinh ra, m\u00e0 ch\u1ec9 xu\u1ea5t hi\u1ec7n trong qu\u00e1 tr\u00ecnh s\u1ed1ng (\u0111\u1ed9t bi\u1ebfn gen somatic) v\u00e0 kh\u00f4ng th\u1ec3 di truy\u1ec1n cho con c\u00e1i. C\u00e1c t\u1ebf b\u00e0o kh\u1ecfe m\u1ea1nh v\u00e0 t\u1ebf b\u00e0o b\u1ec7nh c\u00f9ng t\u1ed3n t\u1ea1i song song (mosaic).<\/p>\n<p>\u0110\u1ed9t bi\u1ebfn gen n\u00e0y th\u01b0\u1eddng n\u1eb1m trong m\u1ed9t \u0111o\u1ea1n \u0111\u1eb7c bi\u1ec7t c\u1ee7a gen, gen PIG-A, v\u00e0 \u1ea3nh h\u01b0\u1edfng \u0111\u1ebfn m\u1ed9t ho\u1eb7c nhi\u1ec1u t\u1ebf b\u00e0o g\u1ed1c t\u1ea1o m\u00e1u trong t\u1ee7y x\u01b0\u01a1ng. Gen n\u00e0y t\u1ea1o ra m\u1ed9t ch\u1ea5t x\u00fac t\u00e1c sinh h\u1ecdc (enzyme), th\u01b0\u1eddng \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng \u0111\u1ec3 s\u1ea3n xu\u1ea5t m\u1ed9t h\u1ec7 th\u1ed1ng neo \u0111\u1eb7c bi\u1ec7t, neo glycosylphosphatidylinositol (neo GPI). Neo n\u00e0y n\u1eb1m tr\u00ean m\u00e0ng t\u1ebf b\u00e0o v\u00e0 c\u00f3 ch\u1ee9c n\u0103ng g\u1eafn k\u1ebft nhi\u1ec1u protein tr\u00ean m\u00e0ng t\u1ebf b\u00e0o, trong \u0111\u00f3 c\u00f3 ch\u1ee9c n\u0103ng \u0111i\u1ec1u h\u00f2a h\u1ec7 mi\u1ec5n d\u1ecbch. B\u1eb1ng c\u00e1ch n\u00e0y, ch\u00fang b\u1ea3o v\u1ec7 c\u00e1c t\u1ebf b\u00e0o kh\u1ecfi s\u1ef1 t\u1ea5n c\u00f4ng c\u1ee7a m\u1ed9t ph\u1ea7n nh\u1ea5t \u0111\u1ecbnh c\u1ee7a h\u1ec7 mi\u1ec5n d\u1ecbch, \u0111\u01b0\u1ee3c g\u1ecdi l\u00e0 <span style=\"color: #000080\"><a style=\"color: #000080\" href=\"#komplsys\">h\u1ec7 th\u1ed1ng b\u1ed5 sung<\/a><\/span>, b\u1eb1ng c\u00e1ch \u0111\u00e1nh d\u1ea5u c\u00e1c t\u1ebf b\u00e0o l\u00e0 &#8220;kh\u00f4ng ph\u1ea3i ngo\u1ea1i lai&#8221;.<\/p>\n<p>Hai trong s\u1ed1 c\u00e1c protein n\u00e0y \u0111\u00f3ng vai tr\u00f2 \u0111\u1eb7c bi\u1ec7t quan tr\u1ecdng:<\/p>\n<ul>\n<li>Y\u1ebfu t\u1ed1 t\u0103ng t\u1ed1c ph\u00e2n h\u1ee7y b\u1ed5 sung (DAF, CD55)<\/li>\n<li>Protectin (MAC-IP: Protein \u1ee9c ch\u1ebf ph\u1ee9c h\u1ee3p t\u1ea5n c\u00f4ng m\u00e0ng, MIRL: Ch\u1ea5t \u1ee9c ch\u1ebf ph\u00e2n h\u1ee7y ph\u1ea3n \u1ee9ng m\u00e0ng, CD59)<\/li>\n<\/ul>\n<p>S\u1ef1 gi\u1ea3m ho\u1eb7c thi\u1ebfu h\u1ee5t ho\u00e0n to\u00e0n c\u00e1c protein g\u1eafn GPI tr\u00ean m\u00e0ng t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u, b\u1ea1ch c\u1ea7u v\u00e0 ti\u1ec3u c\u1ea7u l\u00e0m cho c\u00e1c t\u1ebf b\u00e0o n\u00e0y d\u1ec5 b\u1ecb ph\u00e1 h\u1ee7y b\u1edfi h\u1ec7 th\u1ed1ng b\u1ed5 sung. \u0110i\u1ec1u n\u00e0y d\u1eabn \u0111\u1ebfn s\u1ef1 v\u1ee1 h\u1ed3ng c\u1ea7u PNH trong m\u1ea1ch m\u00e1u (huy\u1ebft t\u00e1n n\u1ed9i m\u1ea1ch) v\u00e0 k\u00edch ho\u1ea1t ti\u1ec3u c\u1ea7u (PNH), c\u00f3 th\u1ec3 d\u1eabn \u0111\u1ebfn huy\u1ebft kh\u1ed1i.<\/p>\n<p><a id=\"komplsys\"><\/a><strong>H\u1ec7 th\u1ed1ng b\u1ed5 sung gi\u00fap c\u01a1 th\u1ec3 ch\u1ed1ng l\u1ea1i c\u00e1c t\u00e1c nh\u00e2n g\u00e2y nhi\u1ec5m tr\u00f9ng, k\u00fd sinh tr\u00f9ng, c\u00e1c ph\u00e2n t\u1eed l\u1ea1, v.v. Khi h\u1ec7 th\u1ed1ng n\u00e0y \u0111\u01b0\u1ee3c k\u00edch ho\u1ea1t, m\u1ed9t qu\u00e1 tr\u00ecnh li\u00ean ti\u1ebfp nh\u01b0 th\u00e1c n\u01b0\u1edbc (th\u00e1c b\u1ed5 sung) s\u1ebd b\u1eaft \u0111\u1ea7u, c\u00f3 th\u1ec3 d\u1eabn \u0111\u1ebfn s\u1ef1 ph\u00e1 h\u1ee7y t\u1ebf b\u00e0o m\u1ee5c ti\u00eau.<\/strong><\/p>\n<h2><a name=\"pnhsymptome\"><\/a><span style=\"color: #f9a801\">2 Tri\u1ec7u ch\u1ee9ng<\/span><\/h2>\n<p>C\u00e1c tri\u1ec7u ch\u1ee9ng v\u00e0 tri\u1ec7u ch\u1ee9ng c\u1ee7a PNH c\u00f3 th\u1ec3 kh\u00e1c nhau v\u00e0 xu\u1ea5t hi\u1ec7n t\u1ea1m th\u1eddi ho\u1eb7c li\u00ean t\u1ee5c. Ngo\u00e0i ra, khi h\u1ec7 th\u1ed1ng b\u1ed5 sung \u0111\u01b0\u1ee3c k\u00edch ho\u1ea1t do nhi\u1ec5m tr\u00f9ng, mang thai ho\u1eb7c c\u0103ng th\u1eb3ng, c\u00e1c tri\u1ec7u ch\u1ee9ng c\u00f3 th\u1ec3 tr\u1edf n\u00ean tr\u1ea7m tr\u1ecdng h\u01a1n, d\u1eabn \u0111\u1ebfn s\u1ef1 ph\u00e2n h\u1ee7y m\u1ea1nh m\u1ebd c\u1ee7a c\u00e1c t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u (kh\u1ee7ng ho\u1ea3ng tan m\u00e1u). Trong qu\u00e1 tr\u00ecnh \u0111i\u1ec1u tr\u1ecb b\u1eb1ng ch\u1ea5t \u1ee9c ch\u1ebf b\u1ed5 sung, hi\u1ec7n t\u01b0\u1ee3ng n\u00e0y c\u00f2n \u0111\u01b0\u1ee3c g\u1ecdi l\u00e0 tan m\u00e1u \u0111\u1ed9t ph\u00e1. M\u1ed9t d\u1ea5u hi\u1ec7u c\u1ea3nh b\u00e1o \u0111i\u1ec3n h\u00ecnh v\u00e0 r\u00f5 r\u00e0ng cho t\u00ecnh tr\u1ea1ng n\u00e0y l\u00e0 n\u01b0\u1edbc ti\u1ec3u c\u00f3 m\u00e0u s\u1eabm ho\u1eb7c \u0111en (hemoglobin ni\u1ec7u). Nguy c\u01a1 huy\u1ebft kh\u1ed1i c\u0169ng t\u0103ng cao trong tr\u01b0\u1eddng h\u1ee3p n\u00e0y.<br \/>\nTrong nh\u1eefng giai \u0111o\u1ea1n n\u00e0y, th\u01b0\u1eddng c\u1ea7n ph\u1ea3i truy\u1ec1n m\u00e1u (huy\u1ebft c\u1ea7u \u0111\u1ecf c\u00f4 \u0111\u1eb7c, EK) ho\u1eb7c c\u00e1c li\u1ec7u ph\u00e1p \u0111i\u1ec1u tr\u1ecb kh\u00e1c.<\/p>\n<p>N\u1ebfu PNH kh\u00f4ng \u0111\u01b0\u1ee3c \u0111i\u1ec1u tr\u1ecb, t\u00ecnh tr\u1ea1ng n\u00e0y c\u00f3 th\u1ec3 \u0111e d\u1ecda t\u00ednh m\u1ea1ng.<\/p>\n<h3><span style=\"color: #f9a801\">2.1 Gi\u1ea3m s\u1ed1 l\u01b0\u1ee3ng t\u1ebf b\u00e0o (cytopenia)<\/span><\/h3>\n<h4><span style=\"color: #f9a801\">Thi\u1ebfu m\u00e1u (anemia)<\/span><\/h4>\n<p>S\u1ef1 ph\u00e2n h\u1ee7y c\u00e1c t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u (tan m\u00e1u) c\u00f3 th\u1ec3 d\u1eabn \u0111\u1ebfn thi\u1ebfu m\u00e1u (anemia) v\u00e0 do \u0111\u00f3 thi\u1ebfu ch\u1ea5t v\u1eadn chuy\u1ec3n oxy (hemoglobin).<\/p>\n<p><strong>C\u00e1c tri\u1ec7u ch\u1ee9ng bao g\u1ed3m:<\/strong><\/p>\n<ul>\n<li>Da nh\u1ee3t nh\u1ea1t (d\u1ea5u hi\u1ec7u kh\u00f4ng \u0111\u1eb7c hi\u1ec7u)<\/li>\n<li>Suy gi\u1ea3m s\u1ee9c kh\u1ecfe, kh\u00f3 t\u1eadp trung, tr\u1ea7m c\u1ea3m, m\u1ec7t m\u1ecfi, ch\u00e2n n\u1eb7ng, nhanh m\u1ec7t<\/li>\n<li>Kh\u00f3 th\u1edf (kh\u00f3 th\u1edf) khi g\u1eafng s\u1ee9c do s\u1ed1 l\u01b0\u1ee3ng ch\u1ea5t v\u1eadn chuy\u1ec3n oxy gi\u1ea3m<\/li>\n<li>Ch\u00f3ng m\u1eb7t, \u00f9 tai, nh\u1ecbp tim nhanh (nh\u1ecbp tim nhanh), \u0111au ng\u1ef1c (\u0111au th\u1eaft ng\u1ef1c), r\u1ed1i lo\u1ea1n th\u1ecb gi\u00e1c<\/li>\n<\/ul>\n<p><strong>T\u00ecnh tr\u1ea1ng thi\u1ebfu m\u00e1u c\u00f3 th\u1ec3 nghi\u00eam tr\u1ecdng \u0111\u1ebfn m\u1ee9c c\u1ea7n ph\u1ea3i truy\u1ec1n h\u1ed3ng c\u1ea7u (huy\u1ebft s\u1eafc t\u1ed1 c\u00f4 \u0111\u1eb7c).<\/strong><\/p>\n<h4><span style=\"color: #f9a801\">C\u00e1c d\u00f2ng t\u1ebf b\u00e0o kh\u00e1c b\u1ecb gi\u1ea3m<\/span><\/h4>\n<p>Ngo\u00e0i d\u00f2ng t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u, c\u00e1c d\u00f2ng t\u1ebf b\u00e0o m\u00e1u kh\u00e1c c\u0169ng c\u00f3 th\u1ec3 b\u1ecb gi\u1ea3m (cytopenia), v\u00ed d\u1ee5 nh\u01b0 ti\u1ec3u c\u1ea7u (thrombocytopenia) ho\u1eb7c b\u1ea1ch c\u1ea7u h\u1ea1t (neutropenia). \u0110i\u1ec1u n\u00e0y th\u01b0\u1eddng l\u00e0 bi\u1ec3u hi\u1ec7n c\u1ee7a b\u1ec7nh thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n k\u00e8m theo.<\/p>\n<h3><span style=\"color: #f9a801\">2.2 T\u00e1c \u0111\u1ed9ng c\u1ee7a tan m\u00e1u<\/span><\/h3>\n<p>Khi h\u1ed3ng c\u1ea7u b\u1ecb ph\u00e2n h\u1ee7y, s\u1ef1 ph\u00e2n h\u1ee7y hemoglobin l\u00e0m t\u0103ng l\u01b0\u1ee3ng bilirubin trong m\u00e1u. \u0110i\u1ec1u n\u00e0y c\u00f3 th\u1ec3 d\u1eabn \u0111\u1ebfn da v\u00e0 ph\u1ea7n tr\u1eafng b\u00ean ngo\u00e0i c\u1ee7a nh\u00e3n c\u1ea7u (sklera). T\u00ecnh tr\u1ea1ng n\u00e0y \u0111\u01b0\u1ee3c g\u1ecdi l\u00e0 v\u00e0ng da (ikterus ho\u1eb7c sklerenikterus).<\/p>\n<p>Ngo\u00e0i ra, khi c\u00e1c t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u b\u1ecb ph\u00e2n h\u1ee7y, hemoglobin s\u1ebd \u0111\u01b0\u1ee3c gi\u1ea3i ph\u00f3ng. Khi l\u01b0\u1ee3ng hemoglobin t\u1ef1 do t\u0103ng cao, n\u00f3 c\u00f3 th\u1ec3 \u0111\u01b0\u1ee3c b\u00e0i ti\u1ebft qua th\u1eadn v\u00e0 d\u1eabn \u0111\u1ebfn n\u01b0\u1edbc ti\u1ec3u c\u00f3 m\u00e0u s\u1eabm, n\u00e2u \u0111\u1ecf ho\u1eb7c th\u1eadm ch\u00ed \u0111en (hemoglobinuria).<br \/>\nQua c\u00e1c b\u01b0\u1edbc ph\u00e2n h\u1ee7y kh\u00e1c nhau, hemoglobin t\u1ef1 do d\u1eabn \u0111\u1ebfn gi\u1ea3m s\u1ef1 s\u1eb5n c\u00f3 c\u1ee7a oxit nitric (NO). Oxit nitric c\u1ea7n thi\u1ebft cho s\u1ef1 gi\u00e3n n\u1edf c\u1ee7a c\u01a1 tr\u01a1n, v\u00ed d\u1ee5 nh\u01b0 trong \u0111\u01b0\u1eddng ti\u00eau h\u00f3a, m\u1ea1ch m\u00e1u ho\u1eb7c ph\u1ed5i. N\u1ebfu kh\u00f4ng c\u00f3 \u0111\u1ee7 oxit nitric, c\u01a1 tr\u01a1n s\u1ebd c\u0103ng th\u1eb3ng, d\u1eabn \u0111\u1ebfn co th\u1eaft, co m\u1ea1ch m\u00e1u v\u00e0 t\u0103ng huy\u1ebft \u00e1p.<\/p>\n<p>C\u01a1 ch\u1ebf ho\u1ea1t \u0111\u1ed9ng n\u00e0y gi\u1ea3i th\u00edch nhi\u1ec1u tri\u1ec7u ch\u1ee9ng l\u00e2m s\u00e0ng c\u1ee7a PNH:<\/p>\n<ul>\n<li>\u0110au b\u1ee5ng d\u1eef d\u1ed9i, th\u01b0\u1eddng l\u00e0 \u0111au c\u1ea5p t\u00ednh<\/li>\n<li>Co th\u1eaft th\u1ef1c qu\u1ea3n k\u00e8m theo kh\u00f3 nu\u1ed1t (kh\u00f3 nu\u1ed1t)<\/li>\n<li>Huy\u1ebft \u00e1p cao<\/li>\n<li>\u00c1p l\u1ef1c cao trong tu\u1ea7n ho\u00e0n ph\u1ed5i (t\u0103ng \u00e1p \u0111\u1ed9ng m\u1ea1ch ph\u1ed5i) k\u00e8m theo kh\u00f3 th\u1edf<\/li>\n<li>Suy gi\u1ea3m ch\u1ee9c n\u0103ng th\u1eadn<\/li>\n<li>R\u1ed1i lo\u1ea1n c\u01b0\u01a1ng d\u01b0\u01a1ng (r\u1ed1i lo\u1ea1n ch\u1ee9c n\u0103ng c\u01b0\u01a1ng d\u01b0\u01a1ng)<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<h3><span style=\"color: #f9a801\">2.3 M\u1ec7t m\u1ecfi (Fatigue)<\/span><\/h3>\n<p>M\u1ec7t m\u1ecfi l\u00e0 t\u00ecnh tr\u1ea1ng m\u1ec7t m\u1ecfi ho\u1eb7c ki\u1ec7t s\u1ee9c k\u00e9o d\u00e0i b\u1ea5t th\u01b0\u1eddng, c\u00f3 th\u1ec3 \u1ea3nh h\u01b0\u1edfng \u0111\u00e1ng k\u1ec3 \u0111\u1ebfn kh\u1ea3 n\u0103ng ho\u1ea1t \u0111\u1ed9ng th\u1ec3 ch\u1ea5t v\u00e0 tinh th\u1ea7n. \u0110\u1eb7c \u0111i\u1ec3m c\u1ee7a m\u1ec7t m\u1ecfi l\u00e0 t\u00ecnh tr\u1ea1ng n\u00e0y kh\u00f4ng c\u1ea3i thi\u1ec7n ho\u1eb7c ch\u1ec9 c\u1ea3i thi\u1ec7n kh\u00f4ng \u0111\u00e1ng k\u1ec3 ngay c\u1ea3 khi ngh\u1ec9 ng\u01a1i ho\u1eb7c ng\u1ee7 nhi\u1ec1u h\u01a1n.<\/p>\n<p>M\u1ed9t nguy\u00ean nh\u00e2n c\u00f3 th\u1ec3 g\u00e2y ra m\u1ec7t m\u1ecfi li\u00ean quan \u0111\u1ebfn PNH l\u00e0 thi\u1ebfu h\u1ee5t oxit nitric (NO) do thi\u1ebfu m\u00e1u v\u00e0 s\u1ef1 ph\u00e1 h\u1ee7y c\u00e1c t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u. Tuy nhi\u00ean, m\u1ec7t m\u1ecfi c\u0169ng c\u00f3 th\u1ec3 do c\u00e1c r\u1ed1i lo\u1ea1n kh\u00e1c c\u1ee7a h\u1ec7 th\u1ed1ng mi\u1ec5n d\u1ecbch ho\u1eb7c chuy\u1ec3n h\u00f3a.<\/p>\n<h3><span style=\"color: #f9a801\">2.4 Xu h\u01b0\u1edbng huy\u1ebft kh\u1ed1i (thrombophilia)<\/span><\/h3>\n<p>M\u1ed9t trong nh\u1eefng h\u1eadu qu\u1ea3 nguy hi\u1ec3m nh\u1ea5t c\u1ee7a PNH kh\u00f4ng \u0111\u01b0\u1ee3c \u0111i\u1ec1u tr\u1ecb v\u00e0 s\u1ef1 thi\u1ebfu h\u1ee5t oxit nitric (NO) do \u0111\u00f3 l\u00e0 s\u1ef1 h\u00ecnh th\u00e0nh c\u1ee5c m\u00e1u \u0111\u00f4ng (huy\u1ebft kh\u1ed1i). S\u1ef1 thi\u1ebfu h\u1ee5t n\u00e0y k\u00edch ho\u1ea1t c\u00e1c ti\u1ec3u c\u1ea7u, d\u1eabn \u0111\u1ebfn s\u1ef1 h\u00ecnh th\u00e0nh c\u1ee5c m\u00e1u \u0111\u00f4ng m\u1ed9t c\u00e1ch b\u1ea5t th\u01b0\u1eddng. C\u00e1c c\u1ee5c m\u00e1u \u0111\u00f4ng n\u00e0y c\u00f3 th\u1ec3 l\u00e0m t\u1eafc ngh\u1ebdn c\u00e1c m\u1ea1ch m\u00e1u nh\u1ecf v\u00e0 d\u1eabn \u0111\u1ebfn nh\u1ed3i m\u00e1u \u1edf c\u00e1c c\u01a1 quan b\u1ecb \u1ea3nh h\u01b0\u1edfng. Nhi\u1ec1u b\u1ed9 ph\u1eadn kh\u00e1c nhau c\u1ee7a c\u01a1 th\u1ec3 c\u00f3 th\u1ec3 b\u1ecb \u1ea3nh h\u01b0\u1edfng, v\u00ed d\u1ee5 nh\u01b0 gan ho\u1eb7c n\u00e3o, c\u1ea3 c\u00e1c m\u1ea1ch m\u00e1u d\u1eabn m\u00e1u v\u1ec1 (t\u0129nh m\u1ea1ch) v\u00e0 c\u00e1c m\u1ea1ch m\u00e1u cung c\u1ea5p m\u00e1u (\u0111\u1ed9ng m\u1ea1ch). T\u01b0\u01a1ng t\u1ef1, s\u1ef1 t\u00edch t\u1ee5 cao c\u1ee7a hemoglobin t\u1ef1 do c\u00f3 th\u1ec3 d\u1eabn \u0111\u1ebfn suy th\u1eadn c\u1ea5p t\u00ednh.<\/p>\n<h2><a name=\"pnhdiagnostik\"><\/a><span style=\"color: #f9a801\">3 Ch\u1ea9n \u0111o\u00e1n<\/span><\/h2>\n<p>Ph\u01b0\u01a1ng ph\u00e1p ki\u1ec3m tra \u01b0a th\u00edch \u0111\u1ec3 ch\u1ea9n \u0111o\u00e1n PNH l\u00e0 ph\u01b0\u01a1ng ph\u00e1p \u0111o l\u01b0u l\u01b0\u1ee3ng t\u1ebf b\u00e0o. Ph\u01b0\u01a1ng ph\u00e1p n\u00e0y r\u1ea5t nh\u1ea1y c\u1ea3m v\u00e0 do \u0111\u00f3 c\u00f3 th\u1ec3 ph\u00e1t hi\u1ec7n m\u1ed9t s\u1ed1 l\u01b0\u1ee3ng r\u1ea5t nh\u1ecf c\u00e1c t\u1ebf b\u00e0o b\u1ecb bi\u1ebfn \u0111\u1ed5i b\u1ec7nh l\u00fd. Ngo\u00e0i ra, t\u1ef7 l\u1ec7 t\u1ebf b\u00e0o b\u1ecb \u1ea3nh h\u01b0\u1edfng (k\u00edch th\u01b0\u1edbc klon PNH) v\u00e0 c\u00e1c lo\u1ea1i t\u1ebf b\u00e0o b\u1ecb \u1ea3nh h\u01b0\u1edfng, v\u00ed d\u1ee5 nh\u01b0 h\u1ed3ng c\u1ea7u, b\u1ea1ch c\u1ea7u h\u1ea1t ho\u1eb7c b\u1ea1ch c\u1ea7u \u0111\u01a1n nh\u00e2n, c\u00f3 th\u1ec3 \u0111\u01b0\u1ee3c x\u00e1c \u0111\u1ecbnh r\u1ea5t ch\u00ednh x\u00e1c. \u0110\u1ec3 th\u1ef1c hi\u1ec7n x\u00e9t nghi\u1ec7m n\u00e0y, m\u00e1u \u0111\u01b0\u1ee3c l\u1ea5y t\u1eeb t\u0129nh m\u1ea1ch (m\u00e1u ngo\u1ea1i vi).<br \/>\nKhi ch\u1ea9n \u0111o\u00e1n l\u1ea7n \u0111\u1ea7u, c\u1ea7n th\u1ef1c hi\u1ec7n th\u00eam c\u00e1c x\u00e9t nghi\u1ec7m kh\u00e1c:<\/p>\n<ul>\n<li>Ti\u1ec1n s\u1eed b\u1ec7nh (ti\u1ec1n s\u1eed b\u1ec7nh), bao g\u1ed3m c\u1ea3 ti\u1ec1n s\u1eed gia \u0111\u00ecnh, bao g\u1ed3m c\u00e1c c\u00e2u h\u1ecfi c\u1ee5 th\u1ec3 v\u1ec1 c\u00e1c tri\u1ec7u ch\u1ee9ng \u0111i\u1ec3n h\u00ecnh c\u1ee7a PNH, xem <span style=\"color: #000080\"><a style=\"color: #000080\" href=\"#pnhsymptome\">2 Tri\u1ec7u ch\u1ee9ng<\/a><\/span><\/li>\n<li>Kh\u00e1m s\u1ee9c kh\u1ecfe, ch\u00fa \u00fd c\u00e1c kh\u00eda c\u1ea1nh \u0111\u1eb7c bi\u1ec7t n\u00eau tr\u00ean: d\u1ea5u hi\u1ec7u thi\u1ebfu m\u00e1u, v\u00e0ng da (ikterus), d\u1ea5u hi\u1ec7u huy\u1ebft kh\u1ed1i c\u1ea5p t\u00ednh ho\u1eb7c tr\u01b0\u1edbc \u0111\u00f3, d\u1ea5u hi\u1ec7u ch\u1ea3y m\u00e1u, b\u1ea5t th\u01b0\u1eddng v\u1ec1 th\u1ec3 ch\u1ea5t nh\u01b0 trong tr\u01b0\u1eddng h\u1ee3p thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n b\u1ea9m sinh (xem \u1edf \u0111\u00f3), l\u00e1 l\u00e1ch to (splenomegalia)<\/li>\n<li>Ki\u1ec3m tra t\u1ebf b\u00e0o\n<ul>\n<li style=\"list-style-type: none\">\n<ul>\n<li>H\u00ecnh \u1ea3nh vi th\u1ec3 t\u1ebf b\u00e0o m\u00e1u kh\u00e1c bi\u1ec7t<\/li>\n<li>Ki\u1ec3m tra t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u non, ch\u01b0a tr\u01b0\u1edfng th\u00e0nh trong m\u00e1u (h\u1ed3ng c\u1ea7u l\u01b0\u1edbi)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/li>\n<li>H\u00f3a h\u1ecdc l\u00e2m s\u00e0ng\n<ul>\n<li style=\"list-style-type: none\">\n<ul>\n<li>C\u00e1c th\u00f4ng s\u1ed1 tan m\u00e1u: \u0111\u1eb7c bi\u1ec7t l\u00e0 LDH, haptoglobin, bilirubin<\/li>\n<li>C\u00e1c th\u00f4ng s\u1ed1 ch\u1ee9c n\u0103ng th\u1eadn: creatinine, b\u00e3o h\u00f2a transferrin<\/li>\n<li>M\u1ee9c vitamin B12 v\u00e0 axit folic<\/li>\n<li>T\u00ecnh tr\u1ea1ng s\u1eaft: Ferritin, transferrin, b\u00e3o h\u00f2a transferrin, hemoglobin reticulocyte. V\u1edbi gi\u00e1 tr\u1ecb ferritin &gt; 1000-2000 \u00b5g\/l, t\u00f9y theo di\u1ec5n bi\u1ebfn, c\u1ea7n ti\u1ebfn h\u00e0nh ki\u1ec3m tra c\u00e1c t\u1ed5n th\u01b0\u01a1ng c\u01a1 quan c\u00f3 th\u1ec3 x\u1ea3y ra do qu\u00e1 t\u1ea3i s\u1eaft.<\/li>\n<li>Gi\u00e1 tr\u1ecb BNP trong huy\u1ebft thanh \u0111\u1ec3 \u0111\u00e1nh gi\u00e1 ch\u1ee9c n\u0103ng c\u1ee7a t\u00e2m th\u1ea5t ph\u1ea3i nh\u01b0 m\u1ed9t d\u1ea5u hi\u1ec7u c\u1ee7a t\u0103ng \u00e1p \u0111\u1ed9ng m\u1ea1ch ph\u1ed5i (t\u0103ng huy\u1ebft \u00e1p ph\u1ed5i)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/li>\n<li>Ch\u1ea9n \u0111o\u00e1n ch\u1ee9c n\u0103ng\n<ul>\n<li>Si\u00eau \u00e2m v\u00f9ng b\u1ee5ng (abdomen)<\/li>\n<li>Si\u00eau \u00e2m tim<\/li>\n<li>Ch\u1ee9c n\u0103ng ph\u1ed5i<\/li>\n<li>\u0110i\u1ec7n t\u00e2m \u0111\u1ed3 (EKG)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p>Ch\u1ea9n \u0111o\u00e1n t\u1ee7y x\u01b0\u01a1ng b\u1eb1ng m\u00f4 h\u1ecdc, cytology, cytogenetics v\u00e0 molecular genetics n\u00ean \u0111\u01b0\u1ee3c th\u1ef1c hi\u1ec7n t\u1ea1i th\u1eddi \u0111i\u1ec3m ch\u1ea9n \u0111o\u00e1n ban \u0111\u1ea7u. \u0110i\u1ec1u n\u00e0y \u0111\u1eb7c bi\u1ec7t quan tr\u1ecdng khi \u0111\u1ed3ng th\u1eddi c\u00f3 cytopenia \u1edf m\u1ee9c \u0111\u1ed9 cao \u0111\u1ebfn m\u1ee9c nghi ng\u1edd PNH c\u00f3 li\u00ean quan \u0111\u1ebfn m\u1ed9t b\u1ec7nh huy\u1ebft h\u1ecdc kh\u00e1c, v\u00ed d\u1ee5 nh\u01b0 thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n ho\u1eb7c h\u1ed9i ch\u1ee9ng myelodysplastic (MDS).<br \/>\nKhi ph\u00e1t hi\u1ec7n t\u1ebf b\u00e0o PNH (clone PNH) ho\u1eb7c ch\u1ea9n \u0111o\u00e1n h\u1ed9i ch\u1ee9ng suy t\u1ee7y x\u01b0\u01a1ng (Bone Marrow Failure Syndrome, BMFS), c\u1ea7n ti\u1ebfn h\u00e0nh ki\u1ec3m tra, \u0111\u1eb7c bi\u1ec7t l\u00e0 trong hai n\u0103m \u0111\u1ea7u ti\u00ean sau ch\u1ea9n \u0111o\u00e1n ban \u0111\u1ea7u, c\u1ee9 6 th\u00e1ng m\u1ed9t l\u1ea7n v\u00e0 khi c\u00f3 c\u00e1c tri\u1ec7u ch\u1ee9ng m\u1edbi xu\u1ea5t hi\u1ec7n. T\u1ef7 l\u1ec7 t\u01b0\u01a1ng \u0111\u1ed1i c\u1ee7a PNH-Klon (k\u00edch th\u01b0\u1edbc PNH-Klon) trong t\u1ed5ng s\u1ed1 t\u1ebf b\u00e0o t\u1ee7y x\u01b0\u01a1ng c\u00f3 th\u1ec3 dao \u0111\u1ed9ng v\u00e0 r\u1ea5t quan tr\u1ecdng \u0111\u1ed1i v\u1edbi di\u1ec5n bi\u1ebfn ti\u1ebfp theo c\u1ee7a b\u1ec7nh c\u0169ng nh\u01b0 vi\u1ec7c x\u00e1c \u0111\u1ecbnh v\u00e0 ki\u1ec3m so\u00e1t li\u1ec7u ph\u00e1p \u0111i\u1ec1u tr\u1ecb.<\/p>\n<h2><a name=\"pnhverlauf\"><\/a><span style=\"color: #f9a801\">4 Di\u1ec5n bi\u1ebfn l\u00e2m s\u00e0ng<\/span><\/h2>\n<p>&nbsp;<\/p>\n<h3><span style=\"color: #f9a801\">4.1 T\u1ed5ng quan<\/span><\/h3>\n<p>C\u00e1c tri\u1ec7u ch\u1ee9ng v\u00e0 v\u1ea5n \u0111\u1ec1 c\u1ee7a PNH c\u00f3 th\u1ec3 r\u1ea5t kh\u00e1c nhau v\u00e0 d\u1eabn \u0111\u1ebfn suy gi\u1ea3m ch\u1ea5t l\u01b0\u1ee3ng cu\u1ed9c s\u1ed1ng do c\u00e1c tri\u1ec7u ch\u1ee9ng n\u00eau tr\u00ean.<br \/>\nC\u00e1c c\u01a1 ch\u1ebf c\u1ee7a b\u1ec7nh \u0111\u01b0\u1ee3c m\u00f4 t\u1ea3 c\u00f3 th\u1ec3 d\u1eabn \u0111\u1ebfn t\u0103ng huy\u1ebft \u00e1p, t\u0103ng \u00e1p l\u1ef1c trong tu\u1ea7n ho\u00e0n ph\u1ed5i (t\u0103ng \u00e1p \u0111\u1ed9ng m\u1ea1ch ph\u1ed5i) v\u00e0 r\u1ed1i lo\u1ea1n ch\u1ee9c n\u0103ng th\u1eadn. Nh\u1eefng thay \u0111\u1ed5i n\u00e0y c\u00f3 th\u1ec3 d\u1eabn \u0111\u1ebfn t\u1ed5n th\u01b0\u01a1ng v\u0129nh vi\u1ec5n v\u00e0 c\u1ea7n \u0111\u01b0\u1ee3c theo d\u00f5i li\u00ean t\u1ee5c.<\/p>\n<h3><span style=\"color: #f9a801\">4.2 C\u1ee5c m\u00e1u \u0111\u00f4ng (huy\u1ebft kh\u1ed1i\/thromboembolism)<\/span><\/h3>\n<p>Bi\u1ebfn ch\u1ee9ng nghi\u00eam tr\u1ecdng nh\u1ea5t c\u1ee7a PNH l\u00e0 s\u1ef1 h\u00ecnh th\u00e0nh c\u1ee5c m\u00e1u \u0111\u00f4ng (thrombosis\/thromboembolism), d\u1eabn \u0111\u1ebfn t\u1eafc ngh\u1ebdn m\u1ea1ch m\u00e1u. Theo c\u00e1c nghi\u00ean c\u1ee9u, x\u00e1c su\u1ea5t x\u1ea3y ra thrombosis l\u00e0<\/p>\n<p>&#8211; 30% trong v\u00f2ng 10 n\u0103m n\u1ebfu kh\u00f4ng c\u00f3 li\u1ec7u ph\u00e1p \u0111i\u1ec1u tr\u1ecb c\u1ee5 th\u1ec3 (ch\u1ea5t \u1ee9c ch\u1ebf b\u1ed5 sung, \u1ee9c ch\u1ebf k\u1ebft t\u1eadp ti\u1ec3u c\u1ea7u)<br \/>\n&#8211; 30-50% trong qu\u00e1 tr\u00ecnh b\u1ec7nh n\u1ebfu kh\u00f4ng c\u00f3 bi\u1ec7n ph\u00e1p \u0111i\u1ec1u tr\u1ecb c\u1ee5 th\u1ec3<\/p>\n<p>C\u00e1c bi\u1ebfn ch\u1ee9ng huy\u1ebft kh\u1ed1i t\u1eafc m\u1ea1ch l\u00e0 nguy\u00ean nh\u00e2n g\u00e2y ra t\u1edbi 67% t\u1ed5ng s\u1ed1 ca t\u1eed vong do PNH. Ngay c\u1ea3 nh\u1eefng b\u1ec7nh nh\u00e2n PNH ch\u1ec9 c\u00f3 m\u1ed9t s\u1ed1 \u00edt t\u1ebf b\u00e0o PNH c\u0169ng c\u00f3 nguy c\u01a1 huy\u1ebft kh\u1ed1i cao. Kh\u1ea3 n\u0103ng b\u1ecb huy\u1ebft kh\u1ed1i c\u00f3 th\u1ec3 ph\u1ee5 thu\u1ed9c v\u00e0o k\u00edch th\u01b0\u1edbc c\u1ee7a d\u00f2ng t\u1ebf b\u00e0o PNH.<br \/>\nHuy\u1ebft kh\u1ed1i \u1edf b\u1ec7nh nh\u00e2n PNH xu\u1ea5t hi\u1ec7n \u1edf nh\u1eefng v\u1ecb tr\u00ed \u0111i\u1ec3n h\u00ecnh v\u00e0 \u00edt \u0111i\u1ec3n h\u00ecnh h\u01a1n:<\/p>\n<div id=\"attachment_8988\" style=\"width: 510px\" class=\"wp-caption alignnone\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-8988\" class=\"wp-image-8988 size-large\" src=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/PNHBild1-Viet-500x262.png\" alt=\"\" width=\"500\" height=\"262\" srcset=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/PNHBild1-Viet-500x262.png 500w, https:\/\/aa-pnh.org\/wp-content\/uploads\/PNHBild1-Viet-300x157.png 300w, https:\/\/aa-pnh.org\/wp-content\/uploads\/PNHBild1-Viet-768x402.png 768w, https:\/\/aa-pnh.org\/wp-content\/uploads\/PNHBild1-Viet-700x367.png 700w, https:\/\/aa-pnh.org\/wp-content\/uploads\/PNHBild1-Viet-1100x576.png 1100w, https:\/\/aa-pnh.org\/wp-content\/uploads\/PNHBild1-Viet.png 1314w\" sizes=\"auto, (max-width: 500px) 100vw, 500px\" \/><p id=\"caption-attachment-8988\" class=\"wp-caption-text\"><em>Huy\u1ebft kh\u1ed1i \u1edf b\u1ec7nh nh\u00e2n PNH xu\u1ea5t hi\u1ec7n \u1edf nh\u1eefng v\u1ecb tr\u00ed \u0111i\u1ec3n h\u00ecnh v\u00e0 \u00edt \u0111i\u1ec3n h\u00ecnh h\u01a1n. <\/em><\/p><\/div>\n<h3><span style=\"color: #f9a801\">4.3 R\u1ed1i lo\u1ea1n ch\u1ee9c n\u0103ng th\u1eadn<\/span><\/h3>\n<p>Hai ph\u1ea7n ba s\u1ed1 b\u1ec7nh nh\u00e2n PNH b\u1ecb suy gi\u1ea3m ch\u1ee9c n\u0103ng th\u1eadn. Ch\u1ee9c n\u0103ng l\u1ecdc b\u1ecb suy gi\u1ea3m l\u00e0 nguy\u00ean nh\u00e2n ch\u00ednh d\u1eabn \u0111\u1ebfn s\u1ef1 suy gi\u1ea3m kh\u1ea3 n\u0103ng l\u1ecdc m\u00e1u c\u1ee7a th\u1eadn. Ch\u1ec9 s\u1ed1 \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng \u0111\u1ec3 \u0111\u00e1nh gi\u00e1 ch\u1ee9c n\u0103ng th\u1eadn l\u00e0 \u0111\u1ed9 thanh th\u1ea3i creatinine, cho bi\u1ebft th\u1eddi gian c\u1ea7n thi\u1ebft \u0111\u1ec3 l\u1ecdc creatinine, m\u1ed9t s\u1ea3n ph\u1ea9m ph\u00e2n h\u1ee7y c\u1ee7a c\u01a1, kh\u1ecfi huy\u1ebft t\u01b0\u01a1ng. C\u1ea3 ch\u1ee9c n\u0103ng l\u1ecdc v\u00e0 \u0111\u1ed9 thanh th\u1ea3i creatinine \u0111\u1ec1u c\u00f3 th\u1ec3 suy gi\u1ea3m trong qu\u00e1 tr\u00ecnh ti\u1ebfn tri\u1ec3n c\u1ee7a b\u1ec7nh.<\/p>\n<h3><span style=\"color: #f9a801\">4.4 Gi\u1ea3m s\u1ed1 l\u01b0\u1ee3ng t\u1ebf b\u00e0o (cytopenia)<\/span><\/h3>\n<p>S\u1ef1 gi\u1ea3m r\u00f5 r\u1ec7t s\u1ed1 l\u01b0\u1ee3ng t\u1ebf b\u00e0o m\u00e1u do b\u1ec7nh t\u1ee7y x\u01b0\u01a1ng k\u00e8m theo l\u00e0 nguy\u00ean nh\u00e2n th\u1ee9 hai g\u00e2y ra c\u00e1c bi\u1ebfn ch\u1ee9ng nghi\u00eam tr\u1ecdng, chi\u1ebfm 20% c\u00e1c tr\u01b0\u1eddng h\u1ee3p. Kho\u1ea3ng 15% b\u1ec7nh nh\u00e2n ph\u00e1t tri\u1ec3n thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n v\u1edbi s\u1ef1 thi\u1ebfu h\u1ee5t c\u1ea3 ba d\u00f2ng t\u1ebf b\u00e0o m\u00e1u (pancytopenia). Ng\u01b0\u1ee3c l\u1ea1i, thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n c\u0169ng c\u00f3 th\u1ec3 xu\u1ea5t hi\u1ec7n tr\u01b0\u1edbc, sau \u0111\u00f3 PNH m\u1edbi xu\u1ea5t hi\u1ec7n.<\/p>\n<h2><a name=\"pnhtherapie\"><\/a><span style=\"color: #f9a801\">5 \u0110i\u1ec1u tr\u1ecb<\/span><\/h2>\n<p>&nbsp;<\/p>\n<h3><span style=\"color: #f9a801\">5.1 T\u1ed5ng quan<\/span><\/h3>\n<p>Vi\u1ec7c \u0111i\u1ec1u tr\u1ecb PNH ph\u1ee5 thu\u1ed9c v\u00e0o m\u1ee9c \u0111\u1ed9 nghi\u00eam tr\u1ecdng c\u1ee7a c\u00e1c tri\u1ec7u ch\u1ee9ng v\u00e0 bi\u1ec3u hi\u1ec7n. N\u1ebfu kh\u00f4ng c\u00f3 tri\u1ec7u ch\u1ee9ng \u0111\u00e1ng k\u1ec3, ch\u1ec9 c\u1ea7n theo d\u00f5i ch\u1eb7t ch\u1ebd l\u00e0 \u0111\u1ee7. C\u00f3 th\u1ec3 xem x\u00e9t c\u00e1c bi\u1ec7n ph\u00e1p h\u1ed7 tr\u1ee3 v\u00e0 s\u1eed d\u1ee5ng thu\u1ed1c \u1ee9c ch\u1ebf \u0111\u00f4ng m\u00e1u (ch\u1ed1ng \u0111\u00f4ng m\u00e1u), th\u01b0\u1eddng \u0111\u01b0\u1ee3c g\u1ecdi l\u00e0 thu\u1ed1c l\u00e0m lo\u00e3ng m\u00e1u. Ch\u1ed1ng \u0111\u00f4ng m\u00e1u kh\u00f4ng th\u1ef1c s\u1ef1 l\u00e0m lo\u00e3ng m\u00e1u, m\u00e0 ng\u0103n ng\u1eeba s\u1ef1 t\u1eafc ngh\u1ebdn (&#8220;\u0111\u1eb7c&#8221;) c\u1ee7a m\u1ea1ch m\u00e1u do c\u1ee5c m\u00e1u \u0111\u00f4ng. Trong tr\u01b0\u1eddng h\u1ee3p PNH c\u00f3 tri\u1ec7u ch\u1ee9ng, h\u00ecnh th\u1ee9c \u0111i\u1ec1u tr\u1ecb ph\u1ee5 thu\u1ed9c v\u00e0o m\u1ee9c \u0111\u1ed9 nghi\u00eam tr\u1ecdng c\u1ee7a s\u1ef1 ph\u00e1 h\u1ee7y h\u1ed3ng c\u1ea7u (tan m\u00e1u) v\u00e0 s\u1ef1 hi\u1ec7n di\u1ec7n c\u1ee7a huy\u1ebft kh\u1ed1i ho\u1eb7c c\u1ee5c m\u00e1u \u0111\u00f4ng.<\/p>\n<p>Bi\u1ec3u \u0111\u1ed3 sau \u0111\u00e2y tr\u00ecnh b\u00e0y ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb PNH tan m\u00e1u:<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-8989 size-large\" src=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/PnhBild2-Viet-500x479.png\" alt=\"\" width=\"500\" height=\"479\" srcset=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/PnhBild2-Viet-500x479.png 500w, https:\/\/aa-pnh.org\/wp-content\/uploads\/PnhBild2-Viet-300x287.png 300w, https:\/\/aa-pnh.org\/wp-content\/uploads\/PnhBild2-Viet-768x736.png 768w, https:\/\/aa-pnh.org\/wp-content\/uploads\/PnhBild2-Viet-700x670.png 700w, https:\/\/aa-pnh.org\/wp-content\/uploads\/PnhBild2-Viet-1100x1053.png 1100w, https:\/\/aa-pnh.org\/wp-content\/uploads\/PnhBild2-Viet.png 1324w\" sizes=\"auto, (max-width: 500px) 100vw, 500px\" \/><\/p>\n<p><em>Bi\u1ec3u di\u1ec5n \u0111\u01a1n gi\u1ea3n h\u00f3a c\u1ee7a thu\u1eadt to\u00e1n \u0111i\u1ec1u tr\u1ecb. Thu\u1eadt to\u00e1n \u0111\u1ea7y \u0111\u1ee7 \u0111\u01b0\u1ee3c tr\u00ecnh b\u00e0y trong h\u01b0\u1edbng d\u1eabn c\u1ee7a DGHO tr\u00ean<\/em> <a href=\"https:\/\/www.onkopedia.com\/de\/onkopedia\/guidelines\/paroxysmale-naechtliche-haemoglobinurie-pnh\/@@guideline\/html\/index.html\" target=\"_blank\" rel=\"noopener noreferrer\">Onkopedia<\/a>.<\/p>\n<p>&nbsp;<\/p>\n<h3><span style=\"color: #f9a801\">5.2 \u0110i\u1ec1u tr\u1ecb c\u1ee5 th\u1ec3<\/span><\/h3>\n<p>V\u00ec <span style=\"color: #000080\"><a style=\"color: #000080\" href=\"#komplsys\">h\u1ec7 th\u1ed1ng b\u1ed5 sung<\/a><\/span> V\u00ec h\u1ec7 th\u1ed1ng b\u1ed5 sung (xem 4.1.3) t\u1ea5n c\u00f4ng c\u00e1c t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u PNH b\u1ecb bi\u1ebfn \u0111\u1ed5i b\u1ec7nh l\u00fd, thu\u1ed1c \u0111\u01b0\u1ee3c s\u1eed d\u1ee5ng \u1edf \u0111\u00e2y. Ch\u00fang \u0111\u01b0\u1ee3c g\u1ecdi l\u00e0 ch\u1ea5t \u1ee9c ch\u1ebf b\u1ed5 sung v\u00e0 \u1ee9c ch\u1ebf m\u1ed9t ph\u1ea7n c\u1ee7a h\u1ec7 th\u1ed1ng b\u1ed5 sung. Do \u0111\u00f3, ch\u00fang \u1ee9c ch\u1ebf ho\u1eb7c ng\u0103n ch\u1eb7n ho\u00e0n to\u00e0n s\u1ef1 ph\u00e1 h\u1ee7y c\u00e1c t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u. M\u1ee5c ti\u00eau c\u1ee7a li\u1ec7u ph\u00e1p s\u1eed d\u1ee5ng ch\u1ea5t \u1ee9c ch\u1ebf b\u1ed5 sung l\u00e0 tr\u00e1nh c\u00e1c bi\u1ebfn ch\u1ee9ng nghi\u00eam tr\u1ecdng, \u0111e d\u1ecda t\u00ednh m\u1ea1ng v\u00e0 c\u00e1c t\u1ed5n th\u01b0\u01a1ng c\u00f3 th\u1ec3 x\u1ea3y ra.<br \/>\n<strong>C\u00e1c l\u00fd do y t\u1ebf \u0111\u1ec3 \u0111i\u1ec1u tr\u1ecb b\u1eb1ng Eculizumab\/Ravulizumab bao g\u1ed3m<\/strong><\/p>\n<ul>\n<li>C\u1ee5c m\u00e1u \u0111\u00f4ng<\/li>\n<li>\u0110au b\u1ee5ng c\u1ea5p t\u00ednh<\/li>\n<li>C\u00e1c c\u01a1n tan m\u00e1u<\/li>\n<li>R\u1ed1i lo\u1ea1n ch\u1ee9c n\u0103ng th\u1eadn do PNH<\/li>\n<li>C\u1ea7n truy\u1ec1n m\u00e1u<\/li>\n<li>T\u0103ng \u00e1p l\u1ef1c \u0111\u1ed9ng m\u1ea1ch ph\u1ed5i (t\u0103ng huy\u1ebft \u00e1p ph\u1ed5i) li\u00ean quan \u0111\u1ebfn PNH<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<ul>\n<li style=\"list-style-type: none\">\n<ul>\n<li style=\"list-style-type: none\"><\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p><strong>\u1ee8c ch\u1ebf b\u1ed5 sung C5<\/strong><\/p>\n<p>C\u00e1c li\u1ec7u ph\u00e1p \u0111i\u1ec1u tr\u1ecb PNH c\u00f3 m\u1ee5c ti\u00eau \u0111\u01b0\u1ee3c ph\u00ea duy\u1ec7t l\u00e2u nh\u1ea5t l\u00e0 kh\u00e1ng th\u1ec3 ch\u1ed1ng C5 Eculizumab (Soliris\u00ae) \u0111\u01b0\u1ee3c gi\u1edbi thi\u1ec7u v\u00e0o n\u0103m 2007 v\u00e0 d\u1ea1ng t\u00e1c d\u1ee5ng d\u00e0i Ravulizumab (Ultomiris\u00ae) \u0111\u01b0\u1ee3c ph\u00ea duy\u1ec7t v\u00e0o n\u0103m 2019.<br \/>\nHai ho\u1ea1t ch\u1ea5t n\u00e0y ng\u0103n ch\u1eb7n ho\u1ea1t \u0111\u1ed9ng c\u1ee7a protein C5 trong h\u1ec7 th\u1ed1ng b\u1ed5 sung (\u0111\u01b0\u1ee3c g\u1ecdi l\u00e0 \u1ee9c ch\u1ebf b\u1ed5 sung cu\u1ed1i c\u00f9ng). C\u00e1c nghi\u00ean c\u1ee9u kh\u00e1c nhau \u0111\u00e3 ch\u1ec9 ra r\u1eb1ng Eculizumab v\u00e0 Ravulizumab l\u00e0m gi\u1ea3m \u0111\u00e1ng k\u1ec3 s\u1ef1 ph\u00e1 h\u1ee7y tr\u1ef1c ti\u1ebfp c\u00e1c t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u trong m\u1ea1ch m\u00e1u (huy\u1ebft t\u00e1n n\u1ed9i m\u1ea1ch) nh\u1edd s\u1ef1 ng\u0103n ch\u1eb7n n\u00e0y.<br \/>\nK\u1ebft qu\u1ea3 l\u00e0 ch\u1ea5t l\u01b0\u1ee3ng cu\u1ed9c s\u1ed1ng c\u1ee7a c\u00e1c b\u1ec7nh nh\u00e2n PNH \u0111\u01b0\u1ee3c \u0111i\u1ec1u tr\u1ecb b\u1eb1ng Eculizumab\/Ravulizumab \u0111\u01b0\u1ee3c c\u1ea3i thi\u1ec7n \u0111\u00e1ng k\u1ec3. \u0110i\u1ec1u n\u00e0y th\u1eadm ch\u00ed c\u00f2n \u0111\u01b0\u1ee3c quan s\u00e1t th\u1ea5y \u1edf nh\u1eefng b\u1ec7nh nh\u00e2n kh\u00f4ng c\u00f3 s\u1ef1 b\u00ecnh th\u01b0\u1eddng h\u00f3a n\u1ed3ng \u0111\u1ed9 hemoglobin. \u0110i\u1ec1u n\u00e0y cho th\u1ea5y r\u1eb1ng ngo\u00e0i vi\u1ec7c c\u1ea3i thi\u1ec7n t\u00ecnh tr\u1ea1ng thi\u1ebfu m\u00e1u, m\u1ed9t ph\u1ea7n t\u00e1c d\u1ee5ng c\u1ee7a thu\u1ed1c l\u00e0 do ng\u0103n ch\u1eb7n s\u1ef1 ph\u00e1 h\u1ee7y h\u1ed3ng c\u1ea7u (huy\u1ebft t\u00e1n).<br \/>\nLi\u1ec7u ph\u00e1p \u0111\u1eb7c hi\u1ec7u n\u00e0y \u0111\u00e3 l\u00e0m gi\u1ea3m \u0111\u00e1ng k\u1ec3 nhu c\u1ea7u truy\u1ec1n m\u00e1u, thi\u1ebfu m\u00e1u, m\u1ec7t m\u1ecfi (m\u1ec7t m\u1ecfi), \u0111au b\u1ee5ng c\u1ea5p t\u00ednh, c\u0169ng nh\u01b0 gi\u1ea3m huy\u1ebft \u00e1p cao (t\u0103ng huy\u1ebft \u00e1p \u0111\u1ed9ng m\u1ea1ch) v\u00e0 huy\u1ebft \u00e1p cao \u1edf ph\u1ed5i (t\u0103ng huy\u1ebft \u00e1p ph\u1ed5i). Ngo\u00e0i ra, c\u00e1c nghi\u00ean c\u1ee9u c\u00f2n m\u00f4 t\u1ea3 s\u1ef1 c\u1ea3i thi\u1ec7n ho\u1eb7c th\u1eadm ch\u00ed b\u00ecnh th\u01b0\u1eddng h\u00f3a ch\u1ee9c n\u0103ng th\u1eadn sau khi c\u00f3 th\u1ec3 \u0111\u00e3 b\u1ecb r\u1ed1i lo\u1ea1n ch\u1ee9c n\u0103ng th\u1eadn tr\u01b0\u1edbc \u0111\u00f3.<\/p>\n<div id=\"attachment_8990\" style=\"width: 510px\" class=\"wp-caption alignnone\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-8990\" class=\"wp-image-8990 size-large\" src=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/pnhbild3-viet-500x176.png\" alt=\"\" width=\"500\" height=\"176\" srcset=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/pnhbild3-viet-500x176.png 500w, https:\/\/aa-pnh.org\/wp-content\/uploads\/pnhbild3-viet-300x106.png 300w, https:\/\/aa-pnh.org\/wp-content\/uploads\/pnhbild3-viet-768x270.png 768w, https:\/\/aa-pnh.org\/wp-content\/uploads\/pnhbild3-viet-700x246.png 700w, https:\/\/aa-pnh.org\/wp-content\/uploads\/pnhbild3-viet-1100x387.png 1100w, https:\/\/aa-pnh.org\/wp-content\/uploads\/pnhbild3-viet.png 1318w\" sizes=\"auto, (max-width: 500px) 100vw, 500px\" \/><p id=\"caption-attachment-8990\" class=\"wp-caption-text\">T\u00e1c d\u1ee5ng c\u1ee7a Eculizumab trong \u0111i\u1ec1u tr\u1ecb PNH<\/p><\/div>\n<p>Eculizumab ho\u1eb7c Ravulizumab \u0111\u01b0\u1ee3c ti\u00eam v\u00e0o t\u0129nh m\u1ea1ch (ti\u00eam t\u0129nh m\u1ea1ch) v\u00e0 c\u00f3 th\u1ec3 \u0111\u01b0\u1ee3c th\u1ef1c hi\u1ec7n t\u1ea1i ph\u00f2ng kh\u00e1m ngo\u1ea1i tr\u00fa. Th\u00f4ng th\u01b0\u1eddng, Eculizumab \u0111\u01b0\u1ee3c ti\u00eam truy\u1ec1n m\u1ed9t l\u1ea7n m\u1ed7i tu\u1ea7n trong 4 tu\u1ea7n \u0111\u1ea7u ti\u00ean \u0111\u1ec3 \u0111\u1ea1t n\u1ed3ng \u0111\u1ed9 t\u1ed1i \u0111a, sau \u0111\u00f3 ti\u00eam truy\u1ec1n 2 tu\u1ea7n m\u1ed9t l\u1ea7n \u0111\u1ec3 duy tr\u00ec li\u1ec1u l\u01b0\u1ee3ng. Ng\u01b0\u1ee3c l\u1ea1i, Ravulizumab ph\u1ea3i \u0111\u01b0\u1ee3c ti\u00eam truy\u1ec1n 2 l\u1ea7n trong v\u00f2ng 2 tu\u1ea7n \u0111\u1ec3 \u0111\u1ea1t n\u1ed3ng \u0111\u1ed9 t\u1ed1i \u0111a, sau \u0111\u00f3 ch\u1ec9 ti\u00eam truy\u1ec1n 8 tu\u1ea7n m\u1ed9t l\u1ea7n \u0111\u1ec3 duy tr\u00ec li\u1ec1u l\u01b0\u1ee3ng. Kh\u00e1c v\u1edbi Eculizumab, li\u1ec1u l\u01b0\u1ee3ng Ravulizumab ph\u1ee5 thu\u1ed9c v\u00e0o tr\u1ecdng l\u01b0\u1ee3ng c\u01a1 th\u1ec3. Th\u1eddi gian truy\u1ec1n t\u0129nh m\u1ea1ch l\u1ea1i ph\u1ee5 thu\u1ed9c v\u00e0o li\u1ec1u l\u01b0\u1ee3ng.<\/p>\n<p>V\u1edbi Crovalimab (Piasky\u00ae), t\u1eeb n\u0103m 2024, m\u1ed9t lo\u1ea1i kh\u00e1ng th\u1ec3 Anti-C5 kh\u00e1c \u0111\u00e3 c\u00f3 s\u1eb5n, c\u00f3 th\u1ec3 \u0111\u01b0\u1ee3c ti\u00eam d\u01b0\u1edbi da (ti\u00eam d\u01b0\u1edbi da) v\u1edbi li\u1ec1u l\u01b0\u1ee3ng nh\u1ecf h\u01a1n sau khi ti\u00eam t\u0129nh m\u1ea1ch (truy\u1ec1n) m\u1ed9t l\u1ea7n. Trong tr\u01b0\u1eddng h\u1ee3p n\u00e0y, c\u1ea7n ph\u1ea3i \u0111i\u1ec1u tr\u1ecb duy tr\u00ec 4 tu\u1ea7n m\u1ed9t l\u1ea7n.<br \/>\nTrong khi s\u1ef1 ph\u00e1 h\u1ee7y c\u00e1c t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u trong m\u1ea1ch m\u00e1u (tan m\u00e1u n\u1ed9i m\u1ea1ch) \u0111\u01b0\u1ee3c \u1ee9c ch\u1ebf th\u00e0nh c\u00f4ng b\u1eb1ng \u1ee9c ch\u1ebf b\u1ed5 sung cu\u1ed1i c\u00f9ng v\u1edbi Eculizumab\/Ravulizumab\/ Crovalimab, m\u1ed9t ph\u1ea7n b\u1ec7nh nh\u00e2n hi\u1ec7n nay l\u1ea1i b\u1ecb suy gi\u1ea3m ch\u1eadm c\u00e1c h\u1ed3ng c\u1ea7u b\u1ecb bi\u1ebfn \u0111\u1ed5i b\u1ec7nh l\u00fd b\u00ean ngo\u00e0i m\u1ea1ch m\u00e1u (tan m\u00e1u ngo\u00e0i m\u1ea1ch m\u00e1u). Qu\u00e1 tr\u00ecnh n\u00e0y \u0111\u01b0\u1ee3c k\u00edch ho\u1ea1t b\u1edfi s\u1ef1 t\u00edch t\u1ee5 c\u00e1c ti\u1ec1n ch\u1ea5t c\u1ee7a h\u1ec7 th\u1ed1ng b\u1ed5 sung v\u00e0 c\u00f3 th\u1ec3 \u0111\u01b0\u1ee3c ph\u00e1t hi\u1ec7n b\u1eb1ng c\u00e1c x\u00e9t nghi\u1ec7m m\u00e1u \u0111\u1eb7c bi\u1ec7t (bao g\u1ed3m reticulocytes, gi\u00e1 tr\u1ecb bilirubin, x\u00e9t nghi\u1ec7m Coombs). \u1ee8c ch\u1ebf s\u1ef1 ph\u00e1 h\u1ee7y c\u00e1c t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u b\u00ean ngo\u00e0i m\u1ea1ch m\u00e1u l\u00e0 \u0111i\u1ec3m kh\u1edfi \u0111\u1ea7u cho vi\u1ec7c ph\u00e1t tri\u1ec3n c\u00e1c lo\u1ea1i thu\u1ed1c m\u1edbi.<\/p>\n<p><strong>\u1ee8c ch\u1ebf b\u1ed5 sung C3<\/strong><\/p>\n<p>C3-inhibitor (inhibitor b\u1ed5 sung g\u1ea7n) \u1ee9c ch\u1ebf b\u1ed5 sung trong chu\u1ed7i ph\u1ea3n \u1ee9ng s\u1edbm h\u01a1n C5-inhibitor. Do \u0111\u00f3, c\u00f3 th\u1ec3 gi\u1ea3m ho\u1eb7c ng\u0103n ch\u1eb7n s\u1ef1 n\u1ea1p c\u00e1c ti\u1ec1n ch\u1ea5t b\u1ed5 sung v\u00e0o c\u00e1c t\u1ebf b\u00e0o PNH v\u00e0 do \u0111\u00f3 gi\u1ea3m ho\u1eb7c ng\u0103n ch\u1eb7n s\u1ef1 tan m\u00e1u ngo\u00e0i m\u1ea1ch m\u00e1u. C\u00e1c ch\u1ea5t m\u1edbi n\u00e0y bao g\u1ed3m C3-inhibitor Pegcetacoplan, y\u1ebfu t\u1ed1 D Danicopan v\u00e0 ch\u1ea5t \u1ee9c ch\u1ebf y\u1ebfu t\u1ed1 B Iptacopan. Gi\u1ed1ng nh\u01b0 to\u00e0n b\u1ed9 h\u1ec7 th\u1ed1ng b\u1ed5 sung, c\u00e1c y\u1ebfu t\u1ed1 B v\u00e0 D c\u0169ng l\u00e0 c\u00e1c protein.<\/p>\n<p>(H\u00ecnh minh h\u1ecda s\u1ebd \u0111\u01b0\u1ee3c cung c\u1ea5p sau)<\/p>\n<p>Pegcetacoplan (Aspaveli\u00ae) ph\u1ea3i \u0111\u01b0\u1ee3c ti\u00eam d\u01b0\u1edbi da (ti\u00eam d\u01b0\u1edbi da) 2-3 l\u1ea7n\/tu\u1ea7n trong kho\u1ea3ng 1 gi\u1edd.<\/p>\n<p>Danicopan (Voydeya\u00ae) d\u01b0\u1edbi d\u1ea1ng thu\u1ed1c vi\u00ean ph\u1ea3i \u0111\u01b0\u1ee3c d\u00f9ng th\u00eam 150-200 mg 3 l\u1ea7n\/ng\u00e0y b\u00ean c\u1ea1nh li\u1ec7u ph\u00e1p \u0111i\u1ec1u tr\u1ecb b\u1eb1ng Eculizumab ho\u1eb7c Ravulizumab.<\/p>\n<p>Iptacopan (Fabhalta\u00ae) \u0111\u01b0\u1ee3c d\u00f9ng d\u01b0\u1edbi d\u1ea1ng thu\u1ed1c vi\u00ean (vi\u00ean nang) v\u1edbi li\u1ec1u 200 mg 2 l\u1ea7n\/ng\u00e0y nh\u01b0 m\u1ed9t li\u1ec7u ph\u00e1p \u0111\u01a1n tr\u1ecb.<\/p>\n<p>Nh\u00ecn chung, d\u1eef li\u1ec7u t\u1eeb c\u00e1c nghi\u00ean c\u1ee9u ph\u00ea duy\u1ec7t v\u00e0 c\u00e1c \u0111\u00e1nh gi\u00e1 hi\u1ec7n t\u1ea1i v\u1ec1 li\u1ec7u ph\u00e1p d\u00e0i h\u1ea1n cho th\u1ea5y \u0111\u00e2y l\u00e0 c\u00e1c li\u1ec7u ph\u00e1p ho\u1eb7c thu\u1ed1c r\u1ea5t d\u1ec5 dung n\u1ea1p. M\u1ed9t t\u00e1c d\u1ee5ng ph\u1ee5 r\u1ea5t ph\u1ed5 bi\u1ebfn l\u00e0 \u0111au \u0111\u1ea7u, ch\u1ec9 x\u1ea3y ra \u1edf giai \u0111o\u1ea1n \u0111\u1ea7u c\u1ee7a li\u1ec7u ph\u00e1p. C\u00e1c t\u00e1c d\u1ee5ng ph\u1ee5 kh\u00e1c c\u00f3 th\u1ec3 x\u1ea3y ra bao g\u1ed3m nhi\u1ec5m tr\u00f9ng, thay \u0111\u1ed5i c\u00f4ng th\u1ee9c m\u00e1u, m\u1ea5t ng\u1ee7, r\u1ed1i lo\u1ea1n ti\u00eau h\u00f3a, v\u1ea5n \u0111\u1ec1 v\u1ec1 da, c\u00e1c tri\u1ec7u ch\u1ee9ng gi\u1ed1ng c\u00fam v\u00e0 m\u1ec7t m\u1ecfi.<\/p>\n<p>Ngo\u00e0i ra, m\u1ed9t \u0111i\u1ec1u \u0111\u00e1ng ch\u00fa \u00fd l\u00e0 c\u00e1c b\u1ec7nh nh\u00e2n PNH ph\u00e0n n\u00e0n nhi\u1ec1u h\u01a1n v\u1ec1 \u0111au \u0111\u1ea7u sau khi d\u00f9ng thu\u1ed1c \u1ee9c ch\u1ebf b\u1ed5 sung l\u1ea7n \u0111\u1ea7u ti\u00ean. Tri\u1ec7u ch\u1ee9ng n\u00e0y l\u00e0 d\u1ea5u hi\u1ec7u cho th\u1ea5y thu\u1ed1c c\u00f3 hi\u1ec7u qu\u1ea3 t\u1ed1t. Do s\u1ed1 l\u01b0\u1ee3ng h\u1ed3ng c\u1ea7u b\u1ecb ph\u00e2n h\u1ee7y \u00edt h\u01a1n, n\u00ean l\u01b0\u1ee3ng oxit nitric (NO) c\u00f3 s\u1eb5n l\u1ea1i nhi\u1ec1u h\u01a1n. Do \u0111\u00f3, c\u00e1c m\u1ea1ch m\u00e1u c\u00f3 th\u1ec3 gi\u00e3n n\u1edf, nh\u01b0ng c\u01a1 th\u1ec3 ph\u1ea3i l\u00e0m quen l\u1ea1i v\u1edbi tr\u1ea1ng th\u00e1i b\u00ecnh th\u01b0\u1eddng n\u00e0y.<\/p>\n<p>Do s\u1ef1 ph\u00e2n h\u1ee7y c\u1ee7a c\u00e1c t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u b\u1ecb b\u1ec7nh gi\u1ea3m, l\u01b0\u1ee3ng hemoglobin v\u00e0 do \u0111\u00f3 l\u01b0\u1ee3ng s\u1eaft \u0111\u01b0\u1ee3c b\u00e0i ti\u1ebft qua n\u01b0\u1edbc ti\u1ec3u c\u0169ng gi\u1ea3m ho\u1eb7c kh\u00f4ng c\u00f2n n\u1eefa. V\u1edbi vi\u1ec7c lo\u1ea1i b\u1ecf s\u1ef1 m\u1ea5t s\u1eaft m\u00e3n t\u00ednh qua th\u1eadn, m\u1ed9t s\u1ed1 b\u1ec7nh nh\u00e2n PNH \u0111\u01b0\u1ee3c \u0111i\u1ec1u tr\u1ecb c\u00f3 s\u1ef1 gia t\u0103ng l\u01b0\u1ee3ng s\u1eaft d\u1ef1 tr\u1eef. Do \u0111\u00f3, l\u01b0\u1ee3ng s\u1eaft d\u1ef1 tr\u1eef c\u1ea7n \u0111\u01b0\u1ee3c ki\u1ec3m tra th\u01b0\u1eddng xuy\u00ean, \u0111\u1eb7c bi\u1ec7t l\u00e0 khi k\u00e8m theo thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n, \u0111\u1ec3 c\u00f3 th\u1ec3 ng\u1eebng \u0111i\u1ec1u tr\u1ecb b\u1eb1ng vi\u00ean s\u1eaft k\u1ecbp th\u1eddi (m\u1ee5c ti\u00eau ferritin &gt; 100 \u00b5g\/l) v\u00e0 n\u1ebfu c\u1ea7n thi\u1ebft, lo\u1ea1i b\u1ecf l\u01b0\u1ee3ng s\u1eaft d\u01b0 th\u1eeba b\u1eb1ng li\u1ec7u ph\u00e1p chelat.<\/p>\n<p>Tuy nhi\u00ean, li\u1ec7u ph\u00e1p \u1ee9c ch\u1ebf b\u1ed5 sung kh\u00f4ng l\u00e0m gi\u1ea3m s\u1ed1 l\u01b0\u1ee3ng t\u1ebf b\u00e0o b\u1ec7nh ho\u1eb7c ch\u1eefa kh\u1ecfi b\u1ec7nh. Ng\u01b0\u1ee3c l\u1ea1i, nh\u1edd s\u1ef1 b\u1ea3o v\u1ec7 c\u1ee7a \u1ee9c ch\u1ebf b\u1ed5 sung, \u00edt ho\u1eb7c kh\u00f4ng c\u00f3 t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u b\u1ecb bi\u1ebfn \u0111\u1ed5i b\u1ec7nh l\u00fd b\u1ecb ph\u00e2n h\u1ee7y, do \u0111\u00f3 s\u1ed1 l\u01b0\u1ee3ng t\u1ebf b\u00e0o h\u1ed3ng c\u1ea7u t\u0103ng l\u00ean. S\u1ef1 gia t\u0103ng c\u1ee7a c\u00e1c h\u1ed3ng c\u1ea7u PNH ho\u1eb7c gi\u00e1 tr\u1ecb hemoglobin l\u00e0 d\u1ea5u hi\u1ec7u cho th\u1ea5y hi\u1ec7u qu\u1ea3 c\u1ee7a li\u1ec7u ph\u00e1p. Do \u0111\u00f3, vi\u1ec7c \u0111i\u1ec1u tr\u1ecb th\u01b0\u1eddng xuy\u00ean ho\u1eb7c u\u1ed1ng thu\u1ed1c th\u01b0\u1eddng xuy\u00ean l\u00e0 r\u1ea5t quan tr\u1ecdng \u0111\u1ec3 \u0111\u1ea3m b\u1ea3o b\u1ea3o v\u1ec7 li\u00ean t\u1ee5c c\u00e1c t\u1ebf b\u00e0o b\u1ecb b\u1ec7nh v\u00e0 tr\u00e1nh c\u00e1c bi\u1ebfn ch\u1ee9ng c\u1ee7a PNH (tan m\u00e1u \u0111\u1ed9t ng\u1ed9t) \u0111\u00e3 \u0111\u1ec1 c\u1eadp.<\/p>\n<p><strong>Ti\u00eam ph\u00f2ng<\/strong><\/p>\n<p>Ti\u00eam ph\u00f2ng l\u00e0 c\u1ea7n thi\u1ebft v\u00ec c\u01a1 th\u1ec3 kh\u00f4ng th\u1ec3 b\u1ea3o v\u1ec7 m\u00ecnh \u0111\u1ee7 tr\u01b0\u1edbc c\u00e1c vi khu\u1ea9n c\u00f3 v\u1ecf b\u1ecdc (meningococcus, pneumococcus v\u00e0 haemophilus influenzae) do h\u1ec7 th\u1ed1ng b\u1ed5 sung b\u1ecb \u1ee9c ch\u1ebf. C\u00e1c vi khu\u1ea9n n\u00e0y c\u00f3 th\u1ec3 g\u00e2y ra nhi\u1ec5m tr\u00f9ng nghi\u00eam tr\u1ecdng, v\u00ed d\u1ee5 nh\u01b0 nhi\u1ec5m tr\u00f9ng m\u00e1u, vi\u00eam ph\u1ed5i, vi\u00eam m\u00e0ng n\u00e3o do vi khu\u1ea9n. Do \u0111\u00f3, khi \u0111i\u1ec1u tr\u1ecb b\u1eb1ng Eculizumab\/Ravulizumab\/Crovalimab, vi\u1ec7c ti\u00eam ph\u00f2ng ng\u1eeba vi khu\u1ea9n n\u00e3o m\u00f4 c\u1ea7u l\u00e0 b\u1eaft bu\u1ed9c. C\u1ea7n ph\u1ea3i s\u1eed d\u1ee5ng hai lo\u1ea1i v\u1eafc-xin kh\u00e1c nhau (Menveo\u00ae\/Nimenrix\u00ae v\u00e0 Bexsero\u00ae\/Trumenba\u00ae) \u0111\u1ec3 bao ph\u1ee7 c\u00e0ng nhi\u1ec1u ch\u1ee7ng vi khu\u1ea9n n\u00e3o m\u00f4 c\u1ea7u c\u00e0ng t\u1ed1t (A, C, W135, Y khi b\u1eaft \u0111\u1ea7u \u0111i\u1ec1u tr\u1ecb v\u00e0 B trong qu\u00e1 tr\u00ecnh \u0111i\u1ec1u tr\u1ecb), m\u1eb7c d\u00f9 v\u1eady v\u1eabn kh\u00f4ng th\u1ec3 \u0111\u1ea1t \u0111\u01b0\u1ee3c s\u1ef1 b\u1ea3o v\u1ec7 100%. C\u1ea7n ti\u00eam v\u1eafc-xin ph\u00f2ng ng\u1eeba m\u1ed7i ba n\u0103m m\u1ed9t l\u1ea7n.<\/p>\n<p>Ngo\u00e0i ra, b\u1ec7nh nh\u00e2n \u0111\u01b0\u1ee3c \u0111i\u1ec1u tr\u1ecb b\u1eb1ng Pegcetacoplan ho\u1eb7c Iptacopan c\u00f2n ph\u1ea3i ti\u00eam v\u1eafc-xin ph\u00f2ng ng\u1eeba vi khu\u1ea9n ph\u1ebf c\u1ea7u (Prevenar 20\u00ae) v\u00e0 Haemophilus influenzae lo\u1ea1i B (ActHib\u00ae ho\u1eb7c Hiberix\u00ae). V\u1eafc-xin ph\u00f2ng ng\u1eeba vi khu\u1ea9n ph\u1ebf c\u1ea7u n\u00ean \u0111\u01b0\u1ee3c ti\u00eam l\u1ea1i sau m\u1ed7i 6 n\u0103m. Vaccine ph\u00f2ng ng\u1eeba Haemophilus ch\u1ec9 ti\u00eam m\u1ed9t l\u1ea7n.<\/p>\n<p><strong>M\u1eb7c d\u00f9 \u0111\u00e3 ti\u00eam vaccine, c\u00e1c b\u1ec7nh nhi\u1ec5m tr\u00f9ng nghi\u00eam tr\u1ecdng k\u00e8m theo nhi\u1ec5m tr\u00f9ng m\u00e1u (s\u1ed1c nhi\u1ec5m tr\u00f9ng) v\u1eabn c\u00f3 th\u1ec3 x\u1ea3y ra, do \u0111\u00f3, lu\u00f4n tu\u00e2n th\u1ee7 c\u00e1c quy t\u1eafc h\u00e0nh vi sau:<\/strong><br \/>\n<strong><br \/>\nKhi b\u1ecb s\u1ed1t (nhi\u1ec7t \u0111\u1ed9 &gt; 38,3 \u00b0C), ph\u00e1t ban, c\u1ea3m gi\u00e1c \u1ed1m n\u1eb7ng, c\u1ee9ng c\u1ed5, nhi\u1ec5m tr\u00f9ng \u0111\u01b0\u1eddng h\u00f4 h\u1ea5p n\u1eb7ng, \u0111i\u1ec1u quan tr\u1ecdng l\u00e0 ph\u1ea3i ti\u1ebfn h\u00e0nh &#8220;\u0111i\u1ec1u tr\u1ecb d\u1ef1 ph\u00f2ng&#8221; (v\u00ed d\u1ee5: Amoxicillin\/Clavulanic acid 1000 mg) c\u00e0ng s\u1edbm c\u00e0ng t\u1ed1t v\u00e0 \u0111\u1ebfn g\u1eb7p b\u00e1c s\u0129 ngay l\u1eadp t\u1ee9c \u0111\u1ec3 ti\u1ebfn h\u00e0nh ch\u1ea9n \u0111o\u00e1n th\u00eam v\u00e0 n\u1ebfu c\u1ea7n thi\u1ebft, b\u1eaft \u0111\u1ea7u \u0111i\u1ec1u tr\u1ecb kh\u00e1ng sinh m\u1edf r\u1ed9ng.<\/strong><\/p>\n<p>Ngo\u00e0i ra, c\u1ea7n th\u1ea3o lu\u1eadn v\u1edbi b\u00e1c s\u0129 huy\u1ebft h\u1ecdc \u0111i\u1ec1u tr\u1ecb v\u1ec1 t\u00ecnh tr\u1ea1ng ti\u00eam ch\u1ee7ng hi\u1ec7n t\u1ea1i, \u0111\u1eb7c bi\u1ec7t l\u00e0 v\u1ec1 c\u00e1c lo\u1ea1i v\u1eafc-xin ti\u00eam b\u1ed5 sung nh\u01b0 c\u00fam, COVID-19, virus h\u1ee3p b\u00e0o h\u00f4 h\u1ea5p (RSV), zona (herpes zoster) v\u00e0 v\u1eafc-xin ti\u00eam nh\u1eafc l\u1ea1i.<\/p>\n<p>&nbsp;<\/p>\n<h3><span style=\"color: #f9a801\">5.3 \u0110i\u1ec1u tr\u1ecb tri\u1ec7u ch\u1ee9ng<\/span><\/h3>\n<p>Ngo\u00e0i c\u00e1c ch\u1ea5t \u1ee9c ch\u1ebf b\u1ed5 sung \u0111\u00e3 n\u00eau, c\u00f2n c\u00f3 c\u00e1c ph\u01b0\u01a1ng ph\u00e1p kh\u00e1c \u0111\u1ec3 \u0111i\u1ec1u tr\u1ecb c\u00e1c tri\u1ec7u ch\u1ee9ng chung.<\/p>\n<p>Trong tr\u01b0\u1eddng h\u1ee3p c\u00f3 tri\u1ec7u ch\u1ee9ng thi\u1ebfu m\u00e1u, c\u00f3 th\u1ec3 ti\u00eam huy\u1ebft c\u1ea7u \u0111\u1ecf. M\u1eb7c d\u00f9 truy\u1ec1n m\u00e1u s\u1ebd cung c\u1ea5p m\u1ed9t l\u01b0\u1ee3ng nh\u1ecf c\u00e1c y\u1ebfu t\u1ed1 b\u1ed5 sung, nh\u01b0ng \u0111i\u1ec1u n\u00e0y s\u1ebd kh\u00f4ng l\u00e0m t\u0103ng qu\u00e1 tr\u00ecnh tan m\u00e1u do b\u1ed5 sung. \u0110i\u1ec1u n\u00e0y c\u0169ng \u00e1p d\u1ee5ng cho vi\u1ec7c s\u1eed d\u1ee5ng huy\u1ebft t\u01b0\u01a1ng t\u1eadp trung, xem <a href=\"https:\/\/aa-pnh.org\/erkrankungen\/aplastische-anaemie#tx\">Thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n, 5.5 \u0110i\u1ec1u tr\u1ecb h\u1ed7 tr\u1ee3 &gt; Truy\u1ec1n m\u00e1u<\/a>.<\/p>\n<p>N\u1ebfu PNH kh\u00f4ng li\u00ean quan tr\u1ef1c ti\u1ebfp \u0111\u1ebfn suy t\u1ee7y x\u01b0\u01a1ng, n\u00f3 \u0111\u01b0\u1ee3c g\u1ecdi l\u00e0 &#8220;PNH c\u1ed5 \u0111i\u1ec3n&#8221;. N\u1ebfu kh\u00f4ng \u0111\u01b0\u1ee3c \u0111i\u1ec1u tr\u1ecb, t\u00ecnh tr\u1ea1ng thi\u1ebfu s\u1eaft th\u01b0\u1eddng x\u1ea3y ra do m\u1ea5t hemoglobin li\u00ean t\u1ee5c qua th\u1eadn. V\u00ec s\u1eaft c\u1ea7n thi\u1ebft cho qu\u00e1 tr\u00ecnh h\u00ecnh th\u00e0nh h\u1ed3ng c\u1ea7u, n\u00ean trong nh\u1eefng tr\u01b0\u1eddng h\u1ee3p n\u00e0y, b\u1ec7nh nh\u00e2n ph\u1ea3i \u0111\u01b0\u1ee3c b\u1ed5 sung s\u1eaft. S\u1eaft c\u00f3 th\u1ec3 \u0111\u01b0\u1ee3c b\u1ed5 sung d\u01b0\u1edbi d\u1ea1ng vi\u00ean n\u00e9n ho\u1eb7c truy\u1ec1n t\u0129nh m\u1ea1ch. T\u1ed1t nh\u1ea5t, thu\u1ed1c u\u1ed1ng n\u00ean \u0111\u01b0\u1ee3c d\u00f9ng v\u00e0o bu\u1ed5i s\u00e1ng m\u1ed7i hai ng\u00e0y m\u1ed9t l\u1ea7n khi b\u1ee5ng \u0111\u00f3i. Ngo\u00e0i ra, kh\u00f4ng n\u00ean d\u00f9ng s\u1eaft c\u00f9ng v\u1edbi thu\u1ed1c kh\u00e1ng sinh ho\u1eb7c thu\u1ed1c trung h\u00f2a axit d\u1ea1 d\u00e0y (thu\u1ed1c kh\u00e1ng axit). Khi b\u1eaft \u0111\u1ea7u \u0111i\u1ec1u tr\u1ecb b\u1eb1ng s\u1eaft, c\u1ea7n ki\u1ec3m tra th\u01b0\u1eddng xuy\u00ean quy\u1ebft \u0111\u1ecbnh \u0111i\u1ec1u tr\u1ecb b\u1ed5 sung s\u1eaft. \u0110\u1ec3 l\u00e0m \u0111i\u1ec1u n\u00e0y, c\u1ea7n ki\u1ec3m tra n\u1ed3ng \u0111\u1ed9 ferritin trong c\u00e1c cu\u1ed9c ki\u1ec3m tra y t\u1ebf (m\u1ee5c ti\u00eau ferritin &gt; 100 \u00b5g\/l).<\/p>\n<p>Do s\u1ef1 b\u00f9 \u0111\u1eafp t\u0103ng c\u01b0\u1eddng t\u00e1i t\u1ea1o h\u1ed3ng c\u1ea7u, nhu c\u1ea7u axit folic v\u00e0 vitamin B12 c\u0169ng t\u0103ng l\u00ean. Trong tr\u01b0\u1eddng h\u1ee3p n\u00e0y, n\u00ean b\u1ed5 sung 5 mg axit folic m\u1ed7i ng\u00e0y ho\u1eb7c 1-2 l\u1ea7n\/tu\u1ea7n. N\u1ebfu thi\u1ebfu vitamin B12, n\u00ean b\u1ed5 sung trong m\u1ed9t th\u1eddi gian nh\u1ea5t \u0111\u1ecbnh.<\/p>\n<p>Trong c\u00e1c nghi\u00ean c\u1ee9u tr\u01b0\u1edbc \u0111\u00e2y, ng\u01b0\u1eddi ta \u0111\u00e3 quan s\u00e1t th\u1ea5y r\u1eb1ng nguy c\u01a1 huy\u1ebft kh\u1ed1i c\u00f3 li\u00ean quan \u0111\u1ebfn s\u1ed1 l\u01b0\u1ee3ng t\u1ebf b\u00e0o PNH v\u00e0 m\u1ee9c \u0111\u1ed9 ho\u1ea1t \u0111\u1ed9ng tan m\u00e1u. N\u1ebfu t\u1ef7 l\u1ec7 b\u1ea1ch c\u1ea7u h\u1ea1t b\u1ecb r\u1ed1i lo\u1ea1n GPI cao h\u01a1n 50% v\u00e0\/ho\u1eb7c gi\u00e1 tr\u1ecb LDH cao h\u01a1n 1,5 l\u1ea7n so v\u1edbi gi\u00e1 tr\u1ecb b\u00ecnh th\u01b0\u1eddng, th\u00ec nguy c\u01a1 huy\u1ebft kh\u1ed1i s\u1ebd t\u0103ng l\u00ean \u0111\u00e1ng k\u1ec3. Khi nh\u1eefng b\u1ec7nh nh\u00e2n n\u00e0y \u0111\u01b0\u1ee3c d\u00f9ng thu\u1ed1c ch\u1ed1ng \u0111\u00f4ng m\u00e1u ph\u00f2ng ng\u1eeba, h\u1ecd \u00edt b\u1ecb huy\u1ebft kh\u1ed1i h\u01a1n \u0111\u00e1ng k\u1ec3.<\/p>\n<p><strong>Do \u0111\u00f3, c\u00e1c khuy\u1ebfn ngh\u1ecb sau \u0111\u00e2y \u0111\u01b0\u1ee3c \u00e1p d\u1ee5ng:<\/strong><\/p>\n<ul>\n<li>Vi\u1ec7c s\u1eed d\u1ee5ng thu\u1ed1c ch\u1ed1ng \u0111\u00f4ng m\u00e1u n\u00ean \u0111\u01b0\u1ee3c quy\u1ebft \u0111\u1ecbnh ri\u00eang cho t\u1eebng b\u1ec7nh nh\u00e2n.<\/li>\n<li>Khi \u0111i\u1ec1u tr\u1ecb b\u1eb1ng thu\u1ed1c \u1ee9c ch\u1ebf b\u1ed5 sung, kh\u00f4ng c\u1ea7n thi\u1ebft ph\u1ea3i d\u00f9ng thu\u1ed1c ch\u1ed1ng \u0111\u00f4ng m\u00e1u ph\u00f2ng ng\u1eeba.<\/li>\n<li>N\u1ebfu \u0111\u00e3 b\u1eaft \u0111\u1ea7u \u0111i\u1ec1u tr\u1ecb b\u1eb1ng thu\u1ed1c ch\u1ed1ng \u0111\u00f4ng m\u00e1u ph\u00f2ng ng\u1eeba tr\u01b0\u1edbc khi \u0111i\u1ec1u tr\u1ecb b\u1eb1ng thu\u1ed1c \u1ee9c ch\u1ebf b\u1ed5 sung, c\u00f3 th\u1ec3 xem x\u00e9t ng\u1eebng \u0111i\u1ec1u tr\u1ecb sau khi ho\u1ea1t \u0111\u1ed9ng tan m\u00e1u tr\u1edf l\u1ea1i b\u00ecnh th\u01b0\u1eddng trong qu\u00e1 tr\u00ecnh \u0111i\u1ec1u tr\u1ecb b\u1eb1ng thu\u1ed1c \u1ee9c ch\u1ebf b\u1ed5 sung. Tuy nhi\u00ean, tuy\u1ec7t \u0111\u1ed1i kh\u00f4ng n\u00ean t\u1ef1 \u00fd ng\u1eebng \u0111i\u1ec1u tr\u1ecb.<\/li>\n<li>N\u1ebfu \u0111\u00e3 x\u1ea3y ra huy\u1ebft kh\u1ed1i, c\u1ea7n th\u1ef1c hi\u1ec7n ch\u1ed1ng \u0111\u00f4ng m\u00e1u. Th\u1eddi gian \u0111i\u1ec1u tr\u1ecb ph\u1ee5 thu\u1ed9c v\u00e0o v\u1ecb tr\u00ed huy\u1ebft kh\u1ed1i v\u00e0 di\u1ec5n bi\u1ebfn c\u1ee7a b\u1ec7nh.<\/li>\n<li>Trong c\u00e1c t\u00ecnh hu\u1ed1ng r\u1ee7i ro, v\u00ed d\u1ee5 nh\u01b0 n\u1eb1m li\u1ec7t gi\u01b0\u1eddng, h\u1ea1n ch\u1ebf v\u1eadn \u0111\u1ed9ng trong th\u1eddi gian d\u00e0i (b\u00f3 b\u1ed9t), ph\u1eabu thu\u1eadt ho\u1eb7c \u0111i xe bu\u00fdt ho\u1eb7c m\u00e1y bay trong th\u1eddi gian d\u00e0i (&gt; kho\u1ea3ng 4-6 gi\u1edd), n\u00ean th\u1ef1c hi\u1ec7n ch\u1ed1ng \u0111\u00f4ng m\u00e1u (v\u00ed d\u1ee5: b\u1eb1ng c\u00e1ch ti\u00eam ho\u1eb7c u\u1ed1ng thu\u1ed1c) \u0111\u1ec3 ph\u00f2ng ng\u1eeba, t\u00f9y thu\u1ed9c v\u00e0o gi\u00e1 tr\u1ecb ti\u1ec3u c\u1ea7u hi\u1ec7n t\u1ea1i.<\/li>\n<li>N\u00ean ph\u00e1t hi\u1ec7n s\u1edbm c\u00e1c nhi\u1ec5m tr\u00f9ng do vi khu\u1ea9n v\u00e0 \u0111i\u1ec1u tr\u1ecb tri\u1ec7t \u0111\u1ec3 b\u1eb1ng kh\u00e1ng sinh, v\u00ec nhi\u1ec5m tr\u00f9ng c\u00f3 th\u1ec3 d\u1eabn \u0111\u1ebfn t\u00ecnh tr\u1ea1ng PNH x\u1ea5u \u0111i nghi\u00eam tr\u1ecdng k\u00e8m theo kh\u1ee7ng ho\u1ea3ng tan m\u00e1u.<\/li>\n<li>Trong tr\u01b0\u1eddng h\u1ee3p kh\u1ee7ng ho\u1ea3ng tan m\u00e1u\/tan m\u00e1u \u0111\u1ed9t ng\u1ed9t, c\u1ea7n b\u1ed5 sung \u0111\u1ee7 n\u01b0\u1edbc (hydrat h\u00f3a). N\u1ebfu c\u1ea7n, c\u00f3 th\u1ec3 ph\u1ea3i \u0111i\u1ec1u tr\u1ecb b\u1eb1ng kh\u00e1ng sinh, truy\u1ec1n m\u00e1u v\u00e0 b\u1ed5 sung thu\u1ed1c \u1ee9c ch\u1ebf b\u1ed5 sung, c\u0169ng nh\u01b0 l\u1ecdc m\u00e1u (ch\u1ea1y th\u1eadn) n\u1ebfu ch\u1ee9c n\u0103ng th\u1eadn suy gi\u1ea3m.<\/li>\n<li>Trong nh\u1eefng tr\u01b0\u1eddng h\u1ee3p ngo\u1ea1i l\u1ec7, khi c\u00f3 suy gi\u1ea3m t\u1ee7y x\u01b0\u01a1ng (suy t\u1ee7y x\u01b0\u01a1ng) ho\u1eb7c sau khi ph\u00e1t tri\u1ec3n thi\u1ebfu m\u00e1u do th\u1eadn (th\u1eadn), vi\u1ec7c s\u1eed d\u1ee5ng c\u00e1c y\u1ebfu t\u1ed1 t\u0103ng tr\u01b0\u1edfng t\u1ea1o m\u00e1u (huy\u1ebft h\u1ecdc), v\u00ed d\u1ee5 nh\u01b0 erythropoietin ho\u1eb7c G-CSF, c\u00f3 th\u1ec3 l\u00e0 h\u1eefu \u00edch.<\/li>\n<li>N\u1ebfu suy t\u1ee7y x\u01b0\u01a1ng (aplasia) l\u00e0 v\u1ea5n \u0111\u1ec1 ch\u00ednh so v\u1edbi PNH, c\u00f3 th\u1ec3 c\u1ea7n ph\u1ea3i ti\u1ebfn h\u00e0nh li\u1ec7u ph\u00e1p \u1ee9c ch\u1ebf mi\u1ec5n d\u1ecbch ho\u1eb7c c\u1ea5y gh\u00e9p t\u1ebf b\u00e0o g\u1ed1c ho\u1eb7c t\u1ee7y x\u01b0\u01a1ng, xem <a href=\"https:\/\/aa-pnh.org\/erkrankungen\/aplastische-anaemie#aatherapie\">Thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n, 5 Li\u1ec7u ph\u00e1p<\/a>.<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<h3><span style=\"color: #f9a801\">5.4 Ch\u1eefa kh\u1ecfi<\/span><\/h3>\n<p>Ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb PNH duy nh\u1ea5t c\u00f3 tri\u1ec3n v\u1ecdng ch\u1eefa kh\u1ecfi (ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb tri\u1ec7t \u0111\u1ec3) l\u00e0 c\u1ea5y gh\u00e9p t\u1ee7y x\u01b0\u01a1ng ho\u1eb7c t\u1ebf b\u00e0o g\u1ed1c \u0111\u1ed3ng lo\u1ea1i. Tuy nhi\u00ean, ph\u01b0\u01a1ng ph\u00e1p n\u00e0y \u0111i k\u00e8m v\u1edbi t\u1ef7 l\u1ec7 bi\u1ebfn ch\u1ee9ng (t\u1ef7 l\u1ec7 m\u1eafc b\u1ec7nh) v\u00e0 t\u1eed vong (t\u1ef7 l\u1ec7 t\u1eed vong) \u0111\u00e1ng k\u1ec3. Do \u0111\u00f3, quy\u1ebft \u0111\u1ecbnh \u0111i\u1ec1u tr\u1ecb (ch\u1ec9 \u0111\u1ecbnh) c\u1ea5y gh\u00e9p ph\u1ea3i \u0111\u01b0\u1ee3c \u0111\u01b0a ra m\u1ed9t c\u00e1ch r\u1ea5t nghi\u00eam ng\u1eb7t, \u0111\u1eb7c bi\u1ec7t l\u00e0 k\u1ec3 t\u1eeb khi c\u00f3 s\u1eb5n ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb b\u1eb1ng ch\u1ea5t \u1ee9c ch\u1ebf b\u1ed5 sung.<\/p>\n<p><strong>C\u00e1c l\u00fd do y t\u1ebf \u0111\u1ec3 c\u1ea5y gh\u00e9p t\u1ebf b\u00e0o g\u1ed1c l\u00e0<\/strong><\/p>\n<ul>\n<li>C\u00e1c bi\u1ebfn ch\u1ee9ng huy\u1ebft kh\u1ed1i t\u00e1i ph\u00e1t, \u0111e d\u1ecda t\u00ednh m\u1ea1ng, kh\u00f4ng \u0111\u00e1p \u1ee9ng v\u1edbi c\u00e1c ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb kh\u00e1c<\/li>\n<li>Thi\u1ebfu m\u00e1u tan m\u00e1u r\u1ea5t n\u1eb7ng, kh\u00f4ng th\u1ec3 \u0111i\u1ec1u tr\u1ecb (kh\u00e1ng tr\u1ecb), c\u1ea7n ph\u1ea3i truy\u1ec1n m\u00e1u<\/li>\n<li>S\u1ef1 hi\u1ec7n di\u1ec7n c\u1ee7a m\u1ed9t d\u00f2ng PNH trong b\u1ec7nh thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n (AA) ho\u1eb7c h\u1ed9i ch\u1ee9ng r\u1ed1i lo\u1ea1n t\u1ea1o m\u00e1u (MDS), n\u1ebfu b\u1ec7nh thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n ho\u1eb7c h\u1ed9i ch\u1ee9ng r\u1ed1i lo\u1ea1n t\u1ea1o m\u00e1u \u0111\u00e3 l\u00e0 l\u00fd do \u0111\u1ec3 c\u1ea5y gh\u00e9p<\/li>\n<li>Chuy\u1ec3n sang thi\u1ebfu m\u00e1u b\u1ea5t s\u1ea3n, n\u1ebfu li\u1ec7u ph\u00e1p \u1ee9c ch\u1ebf mi\u1ec5n d\u1ecbch kh\u00f4ng \u0111\u01b0\u1ee3c ch\u1ec9 \u0111\u1ecbnh, ho\u1eb7c sang h\u1ed9i ch\u1ee9ng r\u1ed1i lo\u1ea1n t\u1ea1o m\u00e1u<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<h2><a name=\"pnhprognose\"><\/a><span style=\"color: #f9a801\">6 Ti\u00ean l\u01b0\u1ee3ng<\/span><\/h2>\n<p>Do s\u1ef1 gi\u1ea3m \u0111\u00e1ng k\u1ec3 c\u00e1c s\u1ef1 ki\u1ec7n huy\u1ebft kh\u1ed1i d\u01b0\u1edbi li\u1ec7u ph\u00e1p \u1ee9c ch\u1ebf b\u1ed5 sung, b\u1ec7nh nh\u00e2n \u0111\u01b0\u1ee3c ch\u1ea9n \u0111o\u00e1n PNH c\u00f3 tu\u1ed5i th\u1ecd g\u1ea7n nh\u01b0 t\u01b0\u01a1ng \u0111\u01b0\u01a1ng v\u1edbi d\u00e2n s\u1ed1 b\u00ecnh th\u01b0\u1eddng.<\/p>\n<h2><a name=\"pnhkinderwunsch\"><\/a><span style=\"color: #f9a801\">7 Mong mu\u1ed1n c\u00f3 con\/mang thai<\/span><\/h2>\n<p>Cho \u0111\u1ebfn v\u00e0i n\u0103m tr\u01b0\u1edbc, b\u1ec7nh nh\u00e2n PNH th\u01b0\u1eddng \u0111\u01b0\u1ee3c khuy\u00ean kh\u00f4ng n\u00ean mang thai do th\u01b0\u1eddng xuy\u00ean x\u1ea3y ra c\u00e1c bi\u1ebfn ch\u1ee9ng \u0111e d\u1ecda t\u00ednh m\u1ea1ng c\u1ee7a m\u1eb9 v\u00e0 con. Do li\u1ec7u ph\u00e1p kh\u00e1ng th\u1ec3 l\u00e0m gi\u1ea3m \u0111\u00e1ng k\u1ec3 r\u1ee7i ro n\u00e0y, v\u1ea5n \u0111\u1ec1 k\u1ebf ho\u1ea1ch h\u00f3a gia \u0111\u00ecnh l\u1ea1i tr\u1edf n\u00ean quan tr\u1ecdng. Hi\u1ec7n t\u1ea1i, \u0111\u00e3 c\u00f3 c\u00e1c b\u00e1o c\u00e1o v\u1ec1 thai k\u1ef3 trong qu\u00e1 tr\u00ecnh \u0111i\u1ec1u tr\u1ecb b\u1eb1ng Eculizumab, Ravulizumab v\u00e0 Crovalimab v\u1edbi k\u1ebft qu\u1ea3 r\u1ea5t \u0111\u00e1ng kh\u00edch l\u1ec7, m\u1eb7c d\u00f9 s\u1ed1 l\u01b0\u1ee3ng tr\u01b0\u1eddng h\u1ee3p c\u00f2n h\u1ea1n ch\u1ebf. C\u00e1c tr\u01b0\u1eddng h\u1ee3p mang thai n\u00e0y h\u1ea7u nh\u01b0 kh\u00f4ng c\u00f3 bi\u1ebfn ch\u1ee9ng v\u00e0 t\u1ea5t c\u1ea3 tr\u1ebb em \u0111\u1ec1u kh\u1ecfe m\u1ea1nh. Tuy nhi\u00ean, n\u1ebfu c\u00f3 mong mu\u1ed1n mang thai ho\u1eb7c \u0111ang mang thai, b\u1ec7nh nh\u00e2n n\u00ean li\u00ean h\u1ec7 v\u1edbi m\u1ed9t trung t\u00e2m chuy\u00ean khoa v\u1ec1 huy\u1ebft h\u1ecdc v\u00e0 ph\u1ee5 khoa \u0111\u1ec3 x\u00e1c \u0111\u1ecbnh h\u1ed3 s\u01a1 r\u1ee7i ro c\u00e1 nh\u00e2n c\u1ee7a b\u1ec7nh nh\u00e2n v\u00e0 \u0111i\u1ec1u ch\u1ec9nh li\u1ec1u l\u01b0\u1ee3ng thu\u1ed1c \u1ee9c ch\u1ebf b\u1ed5 sung n\u1ebfu c\u1ea7n thi\u1ebft.<\/p>\n<h2><a name=\"pnhregister\"><\/a><span style=\"color: #f9a801\">8 Tri\u1ec3n v\u1ecdng<\/span><\/h2>\n<p>Hi\u1ec7n t\u1ea1i, c\u00e1c ch\u1ea5t \u1ee9c ch\u1ebf h\u1ec7 th\u1ed1ng b\u1ed5 sung kh\u00e1c \u0111ang \u0111\u01b0\u1ee3c th\u1eed nghi\u1ec7m trong c\u00e1c nghi\u00ean c\u1ee9u l\u00e2m s\u00e0ng.<\/p>\n<h2><a name=\"pnhregister\"><\/a><span style=\"color: #f9a801\">9 \u0110\u0103ng k\u00fd<\/span><\/h2>\n<p>V\u00ec PNH l\u00e0 m\u1ed9t b\u1ec7nh r\u1ea5t hi\u1ebfm g\u1eb7p, th\u00f4ng tin li\u00ean quan \u0111\u1ebfn b\u1ec7nh v\u00e0 \u0111i\u1ec1u tr\u1ecb ch\u1ec9 c\u00f3 th\u1ec3 thu \u0111\u01b0\u1ee3c th\u00f4ng qua vi\u1ec7c ph\u00e2n t\u00edch d\u1eef li\u1ec7u c\u1ee7a c\u00e0ng nhi\u1ec1u b\u1ec7nh nh\u00e2n PNH c\u00e0ng t\u1ed1t tr\u00ean ph\u1ea1m vi qu\u1ed1c t\u1ebf. V\u1edbi m\u1ee5c \u0111\u00edch n\u00e0y, \u0110\u0103ng k\u00fd b\u1ec7nh nh\u00e2n PNH qu\u1ed1c t\u1ebf (PNH Registry) \u0111\u00e3 \u0111\u01b0\u1ee3c th\u00e0nh l\u1eadp v\u00e0 \u0111\u00f3ng c\u1eeda v\u00e0o cu\u1ed1i n\u0103m 2024. \u0110\u1ec3 ti\u1ebfp t\u1ee5c ho\u1ea1t \u0111\u1ed9ng n\u00e0y, \u0111\u1eb7c bi\u1ec7t l\u00e0 v\u1edbi c\u00e1c ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb m\u1edbi, m\u1ed9t nh\u00f3m chuy\u00ean gia PNH qu\u1ed1c t\u1ebf (<a href=\"https:\/\/www.pnhinterestgroup.org\/\" target=\"_blank\" rel=\"noopener\">International PNH Interest Group<\/a>, IPIG) \u0111\u00e3 th\u00e0nh l\u1eadp m\u1ed9t s\u1ed5 \u0111\u0103ng k\u00fd m\u1edbi. K\u1ec3 t\u1eeb n\u0103m 2025, sau khi c\u00f3 s\u1ef1 \u0111\u1ed3ng \u00fd c\u1ee7a b\u1ec7nh nh\u00e2n, s\u1ed5 \u0111\u0103ng k\u00fd n\u00e0y s\u1ebd ghi l\u1ea1i d\u1eef li\u1ec7u v\u1ec1 di\u1ec5n bi\u1ebfn b\u1ec7nh, \u0111i\u1ec1u tr\u1ecb v\u00e0 ch\u1ea5t l\u01b0\u1ee3ng cu\u1ed9c s\u1ed1ng d\u01b0\u1edbi d\u1ea1ng \u1ea9n danh 6 th\u00e1ng m\u1ed9t l\u1ea7n. V\u00ec ch\u1ec9 th\u00f4ng qua nh\u1eefng th\u00f4ng tin nh\u01b0 v\u1eady m\u1edbi c\u00f3 th\u1ec3 thu \u0111\u01b0\u1ee3c nh\u1eefng ki\u1ebfn th\u1ee9c m\u1edbi v\u1ec1 b\u1ec7nh v\u00e0 c\u1ea3i thi\u1ec7n h\u01a1n n\u1eefa ph\u01b0\u01a1ng ph\u00e1p \u0111i\u1ec1u tr\u1ecb, n\u00ean t\u1ea5t c\u1ea3 b\u1ec7nh nh\u00e2n PNH n\u00ean \u0111\u1ed3ng \u00fd cung c\u1ea5p d\u1eef li\u1ec7u c\u1ee7a m\u00ecnh cho s\u1ed5 \u0111\u0103ng k\u00fd.<\/p>\n<p><strong>H\u00c3Y THAM GIA!<\/strong><\/p>\n<p>N\u1ebfu quan t\u00e2m ho\u1eb7c c\u00f3 th\u1eafc m\u1eafc, vui l\u00f2ng g\u1eedi email \u0111\u1ebfn Prof. Dr. med. Alexander R\u00f6th (<a href=\"mailto:alexander.roeth@ukessen.de\">alexander.roeth(at)ukessen.de<\/a>) ho\u1eb7c Prof. Dr. med. Hubert Schrezenmeier (<a href=\"mailto:h.schrezenmeier@blutspende.de\">h.schrezenmeier(at)blutspende.de<\/a>).<\/p>\n","protected":false},"excerpt":{"rendered":"<p>B\u1ec7nh hemoglobin ni\u1ec7u k\u1ecbch ph\u00e1t ban \u0111\u00eam (PNH) 1 PNH l\u00e0 g\u00ec 2 Tri\u1ec7u ch\u1ee9ng 3 Ch\u1ea9n \u0111o\u00e1n 4 Di\u1ec5n bi\u1ebfn l\u00e2m s\u00e0ng 5 \u0110i\u1ec1u tr\u1ecb 6 Ti\u00ean l\u01b0\u1ee3ng 7 Mong mu\u1ed1n c\u00f3 con\/mang thai 8 \u0110\u0103ng k\u00fd &nbsp; 1 PNH l\u00e0 g\u00ec &nbsp; 1.1&#8230;<\/p>\n","protected":false},"author":12,"featured_media":0,"parent":8967,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-8975","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/aa-pnh.org\/vi\/wp-json\/wp\/v2\/pages\/8975","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/aa-pnh.org\/vi\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/aa-pnh.org\/vi\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/aa-pnh.org\/vi\/wp-json\/wp\/v2\/users\/12"}],"replies":[{"embeddable":true,"href":"https:\/\/aa-pnh.org\/vi\/wp-json\/wp\/v2\/comments?post=8975"}],"version-history":[{"count":1,"href":"https:\/\/aa-pnh.org\/vi\/wp-json\/wp\/v2\/pages\/8975\/revisions"}],"predecessor-version":[{"id":8991,"href":"https:\/\/aa-pnh.org\/vi\/wp-json\/wp\/v2\/pages\/8975\/revisions\/8991"}],"up":[{"embeddable":true,"href":"https:\/\/aa-pnh.org\/vi\/wp-json\/wp\/v2\/pages\/8967"}],"wp:attachment":[{"href":"https:\/\/aa-pnh.org\/vi\/wp-json\/wp\/v2\/media?parent=8975"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}