{"id":7133,"date":"2019-08-03T12:53:46","date_gmt":"2019-08-03T10:53:46","guid":{"rendered":"https:\/\/aa-pnh.org\/?page_id=7133"},"modified":"2025-10-19T16:31:16","modified_gmt":"2025-10-19T14:31:16","slug":"pnh","status":"publish","type":"page","link":"https:\/\/aa-pnh.org\/ar\/diseases\/pnh\/","title":{"rendered":"PNH"},"content":{"rendered":"<h1><span style=\"color: #f9a801;\">\u0628\u064a\u0644\u0629 \u0627\u0644\u0647\u064a\u0645\u0648\u063a\u0644\u0648\u0628\u064a\u0646 \u0627\u0644\u0628\u0648\u0644 \u0627\u0644\u0647\u064a\u0645\u0648\u063a\u0644\u0648\u0628\u064a\u0646\u064a \u0627\u0644\u0644\u064a\u0644\u064a \u0627\u0644\u0627\u0646\u062a\u064a\u0627\u0628\u064a (PNH)<\/span><\/h1>\n<h4><a href=\"#pnhwasist\">1 \u0645\u0627 \u0647\u0648 PNH<\/a><\/h4>\n<h4><a href=\"#pnhsymptome\">2 \u0627\u0644\u0623\u0639\u0631\u0627\u0636<\/a><\/h4>\n<h4><a href=\"#pnhdiagnostik\">3 \u0627\u0644\u062a\u0634\u062e\u064a\u0635<\/a><\/h4>\n<h4><a href=\"#pnhverlauf\">4 \u0627\u0644\u0645\u0633\u0627\u0631 \u0627\u0644\u0633\u0631\u064a\u0631\u064a<\/a><\/h4>\n<h4><a href=\"#pnhtherapie\">5 \u0627\u0644\u0639\u0644\u0627\u062c<\/a><\/h4>\n<h4><a href=\"#pnhprognose\">6 \u0627\u0644\u062a\u0634\u062e\u064a\u0635<\/a><\/h4>\n<h4><a href=\"#pnhkinderwunsch\">7 \u0627\u0644\u0631\u063a\u0628\u0629 \u0641\u064a \u0625\u0646\u062c\u0627\u0628 \u0627\u0644\u0623\u0637\u0641\u0627\u0644\/\u0627\u0644\u062d\u0645\u0644<\/a><\/h4>\n<h4><a href=\"#pnhregister\">8 \u0627\u0644\u062a\u0633\u062c\u064a\u0644<\/a><\/h4>\n<p>\u0627\u0644\u0646\u0635 \u0627\u0644\u062a\u0627\u0644\u064a \u0647\u0648 \u062a\u0631\u062c\u0645\u0629 \u0622\u0644\u064a\u0629 \u0644\u0644\u0646\u0635 DeepL. \u0646\u0648\u062f \u0623\u0646 \u0646\u0634\u064a\u0631 \u0625\u0644\u0649 \u0623\u0646 \u0627\u0644\u0646\u0635 \u0644\u0627 \u064a\u063a\u0646\u064a \u0639\u0646 \u0627\u0633\u062a\u0634\u0627\u0631\u0629 \u0627\u0644\u0637\u0628\u064a\u0628 \u0648\u0623\u0646\u0647 \u064a\u062c\u0628 \u0639\u0644\u064a\u0643 \u062f\u0627\u0626\u0645\u064b\u0627 \u0627\u0633\u062a\u0634\u0627\u0631\u0629 \u0623\u062e\u0635\u0627\u0626\u064a \u0625\u0646 \u0623\u0645\u0643\u0646.<\/p>\n<h2><a name=\"pnhwasist\"><\/a><span style=\"color: #f9a801;\">1 \u0645\u0627 \u0647\u0648 PNH<\/span><\/h2>\n<h3><span style=\"color: #f9a801;\">1.1 \u0645\u0639\u0644\u0648\u0645\u0627\u062a \u0639\u0627\u0645\u0629<\/span><\/h3>\n<p>\u0645\u062b\u0644 \u0641\u0642\u0631 \u0627\u0644\u062f\u0645 \u0627\u0644\u0644\u0627\u062a\u0646\u0633\u062c\u064a\u060c \u0641\u0625\u0646 \u0628\u064a\u0644\u0629 \u0627\u0644\u0647\u064a\u0645\u0648\u062c\u0644\u0648\u0628\u064a\u0646 \u0641\u064a \u0627\u0644\u062f\u0645 \u0627\u0644\u0627\u0646\u062a\u064a\u0627\u0628\u064a \u0627\u0644\u0644\u064a\u0644\u064a (PNH) \u0644\u064a\u0633 \u0645\u0631\u0636\u0627\u064b \u062e\u0628\u064a\u062b\u0627\u064b\u060c \u0648\u0644\u0643\u0646\u0647 \u0645\u0631\u0636 \u0646\u0627\u062f\u0631 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\u0634\u062e\u0635 \u0633\u0646\u0648\u064a\u0627\u064b. \u0648\u0644\u0643\u0646 \u0646\u0638\u0631\u0627\u064b \u0644\u062a\u0646\u0648\u0639 \u0627\u0644\u0623\u0639\u0631\u0627\u0636\u060c \u064a\u062c\u0628 \u0627\u0641\u062a\u0631\u0627\u0636 \u0623\u0646 \u0647\u0630\u0627 \u0627\u0644\u0645\u0631\u0636 \u0646\u0627\u062f\u0631\u0627\u064b \u0645\u0627 \u064a\u062a\u0645 \u062a\u0634\u062e\u064a\u0635\u0647 \u0644\u0623\u0646\u0647 \u063a\u0627\u0644\u0628\u0627\u064b \u0645\u0627 \u0644\u0627 \u064a\u062a\u0645 \u0627\u0644\u062a\u0639\u0631\u0641 \u0639\u0644\u064a\u0647. \u064a\u062a\u0645 \u062a\u0634\u062e\u064a\u0635 \u0627\u0644\u0645\u0631\u0636 \u0641\u064a \u0623\u063a\u0644\u0628 \u0627\u0644\u0623\u062d\u064a\u0627\u0646 \u0628\u064a\u0646 \u0633\u0646 25 \u064845 \u0639\u0627\u0645\u0627\u064b\u060c \u0648\u064a\u0635\u0627\u0628 \u0628\u0647 \u0643\u0644\u0627 \u0627\u0644\u062c\u0646\u0633\u064a\u0646 \u0628\u0623\u0639\u062f\u0627\u062f \u0645\u062a\u0633\u0627\u0648\u064a\u0629 \u062a\u0642\u0631\u064a\u0628\u0627\u064b. \u0644\u0627 \u064a\u0648\u062c\u062f \u062a\u062c\u0645\u0639 \u0639\u0627\u0626\u0644\u064a.<\/p>\n<h3><span style=\"color: #f9a801;\">1.3 \u0627\u0644\u062a\u0637\u0648\u0631 (\u0627\u0644\u062a\u0633\u0628\u0628)<\/span><\/h3>\n<p>\u064a\u0646\u062c\u0645 \u0641\u0631\u0637 \u062a\u0646\u0633\u062c \u0627\u0644\u062f\u0645 (PNH) \u0639\u0646 \u062a\u063a\u064a\u0631 \u062c\u064a\u0646\u064a (\u0637\u0641\u0631\u0629) \u0641\u064a \u0627\u0644\u062e\u0644\u0627\u064a\u0627 \u0627\u0644\u062c\u0630\u0639\u064a\u0629 \u0627\u0644\u0645\u0643\u0648\u0646\u0629 \u0644\u0644\u062f\u0645 \u0641\u064a \u0646\u062e\u0627\u0639 \u0627\u0644\u0639\u0638\u0645. \u0644\u0627 \u064a\u0648\u062c\u062f \u0647\u0630\u0627 \u0627\u0644\u062a\u063a\u064a\u064a\u0631 \u0645\u0646\u0630 \u0627\u0644\u0648\u0644\u0627\u062f\u0629\u060c \u0648\u0644\u0643\u0646\u0647 \u064a\u062a\u0637\u0648\u0631 \u0641\u0642\u0637 \u062e\u0644\u0627\u0644 \u0627\u0644\u062d\u064a\u0627\u0629 (\u0637\u0641\u0631\u0629 \u062c\u064a\u0646\u064a\u0629 \u062c\u0633\u062f\u064a\u0629) \u0648\u0644\u0627 \u064a\u0645\u0643\u0646 \u0623\u0646 \u064a\u0646\u062a\u0642\u0644 \u0625\u0644\u0649 \u0627\u0644\u0646\u0633\u0644. \u064a\u0645\u0643\u0646 \u0623\u0646 \u062a\u062a\u0639\u0627\u064a\u0634 \u0627\u0644\u062e\u0644\u0627\u064a\u0627 \u0627\u0644\u0633\u0644\u064a\u0645\u0629 \u0648\u0627\u0644\u0645\u0631\u064a\u0636\u0629 \u0641\u064a \u0646\u0641\u0633 \u0627\u0644\u0648\u0642\u062a (\u0641\u0633\u064a\u0641\u0633\u0627\u0621).<\/p>\n<p>\u0639\u0627\u062f\u0629\u064b \u0645\u0627 \u062a\u0642\u0639 \u0647\u0630\u0647 \u0627\u0644\u0637\u0641\u0631\u0629 \u0627\u0644\u062c\u064a\u0646\u064a\u0629 \u0641\u064a \u062c\u0632\u0621 \u0645\u0639\u064a\u0646 \u0645\u0646 \u0627\u0644\u0645\u0627\u062f\u0629 \u0627\u0644\u0648\u0631\u0627\u062b\u064a\u0629\u060c \u0648\u0647\u0648 \u062c\u064a\u0646 PIG-A\u060c \u0648\u062a\u0624\u062b\u0631 \u0639\u0644\u0649 \u0648\u0627\u062d\u062f\u0629 \u0623\u0648 \u0623\u0643\u062b\u0631 \u0645\u0646 \u0627\u0644\u062e\u0644\u0627\u064a\u0627 \u0627\u0644\u062c\u0630\u0639\u064a\u0629 \u0627\u0644\u0645\u0643\u0648\u0646\u0629 \u0644\u0644\u062f\u0645 \u0641\u064a \u0646\u062e\u0627\u0639 \u0627\u0644\u0639\u0638\u0645. \u064a\u064f\u0646\u062a\u062c \u0627\u0644\u062c\u064a\u0646 \u0645\u062d\u0641\u0632\u0627\u064b \u062d\u064a\u0648\u064a\u0627\u064b (\u0625\u0646\u0632\u064a\u0645) \u0645\u0637\u0644\u0648\u0628 \u0639\u0627\u062f\u0629\u064b \u0644\u0625\u0646\u062a\u0627\u062c \u0646\u0638\u0627\u0645 \u062a\u062b\u0628\u064a\u062a \u062e\u0627\u0635\u060c \u0648\u0647\u0648 \u0645\u0631\u0633\u0627\u0629 \u0627\u0644\u063a\u0644\u064a\u0643\u0648\u0632\u064a\u0644\u0641\u0648\u0633\u0641\u0627\u062a\u064a\u062f\u064a\u0644\u064a\u0646\u0648\u0633\u064a\u062a\u0648\u0644 (\u0645\u0631\u0633\u0627\u0629 GPI). \u0648\u064a\u0642\u0639 \u0647\u0630\u0627 \u0627\u0644\u0646\u0638\u0627\u0645 \u0639\u0644\u0649 \u063a\u0634\u0627\u0621 \u0627\u0644\u062e\u0644\u064a\u0629 \u0648\u064a\u0633\u062a\u062e\u062f\u0645 \u0644\u0631\u0628\u0637 \u0627\u0644\u0639\u062f\u064a\u062f \u0645\u0646 \u0627\u0644\u0628\u0631\u0648\u062a\u064a\u0646\u0627\u062a \u0628\u063a\u0634\u0627\u0621 \u0627\u0644\u062e\u0644\u064a\u0629\u060c \u0648\u0627\u0644\u062a\u064a \u062a\u0639\u0645\u0644 \u0639\u0644\u0649 \u062a\u0646\u0638\u064a\u0645 \u0627\u0644\u062c\u0647\u0627\u0632 \u0627\u0644\u0645\u0646\u0627\u0639\u064a\u060c \u0645\u0646 \u0628\u064a\u0646 \u0623\u0645\u0648\u0631 \u0623\u062e\u0631\u0649. \u0648\u0628\u0647\u0630\u0647 \u0627\u0644\u0637\u0631\u064a\u0642\u0629\u060c \u062a\u062d\u0645\u064a \u0647\u0630\u0647 \u0627\u0644\u0628\u0631\u0648\u062a\u064a\u0646\u0627\u062a \u0627\u0644\u062e\u0644\u0627\u064a\u0627 \u0645\u0646 \u0647\u062c\u0648\u0645 \u062c\u0632\u0621 \u0645\u0639\u064a\u0646 \u0645\u0646 \u0627\u0644\u062c\u0647\u0627\u0632 \u0627\u0644\u0645\u0646\u0627\u0639\u064a\u060c \u0648\u0647\u0648 \u0645\u0627 \u064a\u0633\u0645\u0649 <a href=\"#komplsys\">\u0628\u0627\u0644\u062c\u0647\u0627\u0632 \u0627\u0644\u062a\u0643\u0645\u064a\u0644\u064a\u060c<\/a> \u0639\u0644\u0649 \u0633\u0628\u064a\u0644 \u0627\u0644\u0645\u062b\u0627\u0644\u060c \u0639\u0646 \u0637\u0631\u064a\u0642 \u0648\u0633\u0645 \u0627\u0644\u062e\u0644\u0627\u064a\u0627 \u0628\u0623\u0646\u0647\u0627 \u063a\u064a\u0631 \u0623\u062c\u0646\u0628\u064a\u0629.<\/p>\n<p>\u064a\u0644\u0639\u0628 \u0627\u062b\u0646\u0627\u0646 \u0645\u0646 \u0647\u0630\u0647 \u0627\u0644\u0628\u0631\u0648\u062a\u064a\u0646\u0627\u062a \u062f\u0648\u0631\u0627\u064b \u0645\u0647\u0645\u0627\u064b \u0628\u0634\u0643\u0644 \u062e\u0627\u0635 \u0641\u064a \u0647\u0630\u0647 \u0627\u0644\u0639\u0645\u0644\u064a\u0629:<\/p>\n<ul>\n<li>\u0639\u0627\u0645\u0644 \u062a\u0633\u0631\u064a\u0639 \u0627\u0644\u0627\u0636\u0645\u062d\u0644\u0627\u0644 \u0627\u0644\u0645\u0643\u0645\u0644 (DAF\u060c CD55)<\/li>\n<li>\u0628\u0631\u0648\u062a\u0643\u062a\u064a\u0646 (MAC-IP: \u0627\u0644\u0628\u0631\u0648\u062a\u064a\u0646 \u0627\u0644\u0645\u062b\u0628\u0637 \u0644\u0645\u0631\u0643\u0628 \u0647\u062c\u0648\u0645 \u0627\u0644\u063a\u0634\u0627\u0621\u060c MIRL: \u0645\u062b\u0628\u0637 \u0627\u0644\u063a\u0634\u0627\u0621 \u0644\u0644\u062a\u062d\u0644\u0644 \u0627\u0644\u062a\u0641\u0627\u0639\u0644\u064a\u060c CD59)<\/li>\n<\/ul>\n<p>\u0627\u0646\u062e\u0641\u0627\u0636 \u0623\u0648 \u063a\u064a\u0627\u0628 \u0643\u0627\u0645\u0644 \u0644\u0644\u0628\u0631\u0648\u062a\u064a\u0646\u0627\u062a \u0627\u0644\u0645\u062b\u0628\u0651\u062a\u0629 \u0628\u0640 GPI \u0639\u0644\u0649 \u0627\u0644\u063a\u0634\u0627\u0621 \u0627\u0644\u062e\u0644\u0648\u064a \u0644\u0643\u0631\u064a\u0627\u062a \u0627\u0644\u062f\u0645 \u0627\u0644\u062d\u0645\u0631\u0627\u0621 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\u0648\u064a\u064f\u0634\u0627\u0631 \u0625\u0644\u0649 \u0630\u0644\u0643 \u0623\u064a\u0636\u0627\u064b \u0628\u0627\u0644\u064a\u0631\u0642\u0627\u0646 (\u0627\u0644\u064a\u0631\u0642\u0627\u0646 \u0623\u0648 \u0627\u0644\u064a\u0631\u0642\u0627\u0646 \u0627\u0644\u0635\u0641\u0631\u0627\u0648\u064a).<\/p>\n<p>\u0628\u0627\u0644\u0625\u0636\u0627\u0641\u0629 \u0625\u0644\u0649 \u0630\u0644\u0643\u060c \u064a\u062a\u0645 \u0625\u0637\u0644\u0627\u0642 \u0627\u0644\u0647\u064a\u0645\u0648\u063a\u0644\u0648\u0628\u064a\u0646 \u0639\u0646\u062f\u0645\u0627 \u062a\u062a\u0643\u0633\u0631 \u062e\u0644\u0627\u064a\u0627 \u0627\u0644\u062f\u0645 \u0627\u0644\u062d\u0645\u0631\u0627\u0621. \u0625\u0630\u0627 \u0643\u0627\u0646 \u0647\u0646\u0627\u0643 \u0645\u0633\u062a\u0648\u0649 \u0639\u0627\u0644\u064d \u062c\u062f\u0627\u064b \u0645\u0646 \u0627\u0644\u0647\u064a\u0645\u0648\u063a\u0644\u0648\u0628\u064a\u0646 \u0627\u0644\u062d\u0631\u060c \u0641\u064a\u0645\u0643\u0646 \u0623\u0646 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\u0648\u0627\u0644\u064a\u0631\u0642\u0627\u0646 (\u0627\u0644\u064a\u0631\u0642\u0627\u0646)\u060c \u0648\u062f\u0644\u064a\u0644 \u0639\u0644\u0649 \u0648\u062c\u0648\u062f \u062a\u062c\u0644\u0637 \u062d\u0627\u062f \u0623\u0648 \u0633\u0627\u0628\u0642 \u0644\u0644\u062a\u062e\u062b\u0631\u060c \u0648\u0639\u0644\u0627\u0645\u0627\u062a \u0627\u0644\u0646\u0632\u064a\u0641\u060c \u0648\u0627\u0644\u062a\u0634\u0648\u0647\u0627\u062a \u0627\u0644\u062f\u0633\u062a\u0648\u0631\u064a\u0629 \u0645\u062b\u0644 <a href=\"https:\/\/aa-pnh.org\/ar\/erkrankungen\/aplastische-anaemie#aadiagnostik\">\u0641\u0642\u0631 \u0627\u0644\u062f\u0645 \u0627\u0644\u0644\u0627\u062a\u0646\u0633\u062c\u064a<\/a> \u0627\u0644\u062e\u0644\u0642\u064a\u060c \u0648\u062a\u0636\u062e\u0645 \u0627\u0644\u0637\u062d\u0627\u0644 (\u062a\u0636\u062e\u0645 \u0627\u0644\u0637\u062d\u0627\u0644)<\/li>\n<li>\u0641\u062d\u0648\u0635\u0627\u062a \u0627\u0644\u062e\u0644\u0627\u064a\u0627\n<ul>\n<li style=\"list-style-type: none;\">\n<ul>\n<li>\u062a\u0639\u062f\u0627\u062f \u0627\u0644\u062f\u0645 \u0627\u0644\u062a\u0641\u0627\u0636\u0644\u064a \u0627\u0644\u0645\u062c\u0647\u0631\u064a<\/li>\n<li>\u0627\u0644\u062e\u0644\u0627\u064a\u0627 \u0627\u0644\u0634\u0628\u0643\u064a\u0629<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/li>\n<li>\u0627\u0644\u0643\u064a\u0645\u064a\u0627\u0621 \u0627\u0644\u0633\u0631\u064a\u0631\u064a\u0629\n<ul>\n<li style=\"list-style-type: none;\">\n<ul>\n<li>\u0645\u0639\u0627\u064a\u064a\u0631 \u0627\u0646\u062d\u0644\u0627\u0644 \u0627\u0644\u062f\u0645: \u062e\u0627\u0635\u0629\u064b LDH\u060c \u0648\u0627\u0644\u0647\u0627\u0628\u062a\u0648\u063a\u0644\u0648\u0628\u064a\u0646\u060c \u0648\u0627\u0644\u0628\u064a\u0644\u064a\u0631\u0648\u0628\u064a\u0646<\/li>\n<li>\u0645\u0639\u0627\u064a\u064a\u0631 \u0648\u0638\u0627\u0626\u0641 \u0627\u0644\u0643\u0644\u0649: \u0627\u0644\u0643\u0631\u064a\u0627\u062a\u064a\u0646\u064a\u0646\u060c \u062a\u0634\u0628\u0639 \u0627\u0644\u062a\u0631\u0627\u0646\u0633\u0641\u064a\u0631\u064a\u0646<\/li>\n<li>\u0645\u0633\u062a\u0648\u064a\u0627\u062a \u0641\u064a\u062a\u0627\u0645\u064a\u0646 B12 \u0648\u062d\u0645\u0636 \u0627\u0644\u0641\u0648\u0644\u064a\u0643<\/li>\n<li>\u062d\u0627\u0644\u0629 \u0627\u0644\u062d\u062f\u064a\u062f: \u0627\u0644\u0641\u064a\u0631\u064a\u062a\u064a\u0646 \u0648\u0627\u0644\u062a\u0631\u0627\u0646\u0633\u0641\u064a\u0631\u064a\u0646 \u0648\u062a\u0634\u0628\u0639 \u0627\u0644\u062a\u0631\u0627\u0646\u0633\u0641\u064a\u0631\u064a\u0646 \u0648\u0627\u0644\u0647\u064a\u0645\u0648\u062c\u0644\u0648\u0628\u064a\u0646 \u0627\u0644\u0634\u0628\u0643\u064a. \u0625\u0630\u0627 \u0643\u0627\u0646\u062a \u0642\u064a\u0645 \u0627\u0644\u0641\u064a\u0631\u064a\u062a\u064a\u0646 &gt; 1000 \u0645\u064a\u0643\u0631\u0648\u063a\u0631\u0627\u0645\/\u0644\u062a\u0631\u060c \u0641\u064a\u062c\u0628 \u0627\u0644\u062a\u062d\u0642\u0642 \u0645\u0646 \u0627\u062d\u062a\u0645\u0627\u0644 \u062a\u0644\u0641 \u0627\u0644\u0623\u0639\u0636\u0627\u0621 \u0628\u0633\u0628\u0628 \u0627\u0644\u062d\u0645\u0644 \u0627\u0644\u0632\u0627\u0626\u062f \u0644\u0644\u062d\u062f\u064a\u062f.<\/li>\n<li>\u0642\u064a\u0645\u0629 BNP \u0641\u064a \u0645\u0635\u0644 \u0627\u0644\u062f\u0645 \u0644\u062a\u0642\u064a\u064a\u0645 \u0648\u0638\u064a\u0641\u0629 \u0627\u0644\u0628\u0637\u064a\u0646 \u0627\u0644\u0623\u064a\u0645\u0646<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/li>\n<li>\u0627\u0644\u062a\u0634\u062e\u064a\u0635 \u0627\u0644\u0648\u0638\u064a\u0641\u064a\n<ul>\n<li>\u0627\u0644\u0645\u0648\u062c\u0627\u062a \u0641\u0648\u0642 \u0627\u0644\u0635\u0648\u062a\u064a\u0629 \u0644\u0644\u0628\u0637\u0646<\/li>\n<li>\u0648\u0638\u064a\u0641\u0629 \u0627\u0644\u0631\u0626\u0629<\/li>\n<li>\u062a\u062e\u0637\u064a\u0637 \u0627\u0644\u0642\u0644\u0628<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p>\u064a\u062c\u0628 \u0625\u062c\u0631\u0627\u0621 \u062a\u0634\u062e\u064a\u0635 \u0646\u062e\u0627\u0639 \u0627\u0644\u0639\u0638\u0645 \u0645\u0639 \u0639\u0644\u0645 \u0627\u0644\u062e\u0644\u0627\u064a\u0627 \u0648\u0639\u0644\u0645 \u0627\u0644\u0648\u0631\u0627\u062b\u0629 \u0627\u0644\u062e\u0644\u0648\u064a\u0629 \u0648\u0639\u0644\u0645 \u0627\u0644\u0623\u0646\u0633\u062c\u0629 \u0641\u064a \u0648\u0642\u062a \u0627\u0644\u062a\u0634\u062e\u064a\u0635 \u0627\u0644\u0623\u0648\u0644\u064a. \u0647\u0630\u0627 \u0645\u0647\u0645 \u0628\u0634\u0643\u0644 \u062e\u0627\u0635 \u0625\u0630\u0627 \u0643\u0627\u0646 \u0641\u0642\u0631 \u0627\u0644\u062f\u0645 \u0627\u0644\u062e\u0644\u0648\u064a \u0634\u062f\u064a\u062f\u064b\u0627 \u0644\u062f\u0631\u062c\u0629 \u0627\u0644\u0627\u0634\u062a\u0628\u0627\u0647 \u0641\u064a \u0623\u0646 \u0641\u0631\u0637 \u062a\u0646\u0633\u062c \u0627\u0644\u0646\u062e\u0627\u0639 \u0627\u0644\u0634\u0648\u0643\u064a (PNH) \u0645\u0631\u062a\u0628\u0637 \u0628\u0645\u0631\u0636 \u062f\u0645\u0648\u064a \u0622\u062e\u0631\u060c \u0645\u062b\u0644 \u0641\u0642\u0631 \u0627\u0644\u062f\u0645 \u0627\u0644\u0644\u0627\u062a\u0646\u0633\u062c\u064a \u0623\u0648 \u0645\u062a\u0644\u0627\u0632\u0645\u0629 \u062e\u0644\u0644 \u0627\u0644\u062a\u0646\u0633\u062c \u0627\u0644\u0646\u0642\u0648\u064a (MDS).<\/p>\n<p>\u0625\u0630\u0627 \u062a\u0645 \u0627\u0643\u062a\u0634\u0627\u0641 \u062e\u0644\u0627\u064a\u0627 PNH (\u0645\u0633\u062a\u0646\u0633\u062e PNH) \u0623\u0648 \u062a\u0645 \u062a\u0634\u062e\u064a\u0635 \u0645\u062a\u0644\u0627\u0632\u0645\u0629 \u0641\u0634\u0644 \u0646\u062e\u0627\u0639 \u0627\u0644\u0639\u0638\u0645\u060c \u064a\u062c\u0628 \u0625\u062c\u0631\u0627\u0621 \u0641\u062d\u0648\u0635\u0627\u062a \u0643\u0644 6 \u0623\u0634\u0647\u0631\u060c \u062e\u0627\u0635\u0629\u064b \u0641\u064a \u0623\u0648\u0644 \u0639\u0627\u0645\u064a\u0646 \u0628\u0639\u062f \u0627\u0644\u062a\u0634\u062e\u064a\u0635 \u0627\u0644\u0623\u0648\u0644\u064a\u060c \u0648\u0625\u0630\u0627 \u0638\u0647\u0631\u062a \u0623\u0639\u0631\u0627\u0636 \u062c\u062f\u064a\u062f\u0629. \u064a\u0645\u0643\u0646 \u0623\u0646 \u062a\u062a\u0630\u0628\u0630\u0628 \u0627\u0644\u0646\u0633\u0628\u0629 \u0627\u0644\u0646\u0633\u0628\u064a\u0629 \u0644\u0645\u0633\u062a\u0646\u0633\u062e PNH (\u062d\u062c\u0645 \u0645\u0633\u062a\u0646\u0633\u062e PNH) \u0625\u0644\u0649 \u0627\u0644\u0639\u062f\u062f \u0627\u0644\u0625\u062c\u0645\u0627\u0644\u064a \u0644\u062e\u0644\u0627\u064a\u0627 \u0646\u062e\u0627\u0639 \u0627\u0644\u0639\u0638\u0645\u060c \u0648\u0647\u064a \u0645\u0647\u0645\u0629 \u0644\u0644\u0645\u0633\u0627\u0631 \u0627\u0644\u0644\u0627\u062d\u0642 \u0644\u0644\u0645\u0631\u0636 \u0648\u0644\u062a\u062d\u062f\u064a\u062f \u0627\u0644\u0639\u0644\u0627\u062c \u0648\u0645\u0631\u0627\u0642\u0628\u062a\u0647.<\/p>\n<h2><a name=\"pnhverlauf\"><\/a><span style=\"color: #f9a801;\">4 \u0627\u0644\u0645\u0633\u0627\u0631 \u0627\u0644\u0633\u0631\u064a\u0631\u064a<\/span><\/h2>\n<h3><span style=\"color: #f9a801;\">4.1 \u0645\u0639\u0644\u0648\u0645\u0627\u062a \u0639\u0627\u0645\u0629<\/span><\/h3>\n<p>\u064a\u0645\u0643\u0646 \u0623\u0646 \u062a\u062a\u0641\u0627\u0648\u062a \u0623\u0639\u0631\u0627\u0636 \u0648\u0645\u0634\u0627\u0643\u0644 \u0645\u062a\u0644\u0627\u0632\u0645\u0629 \u0641\u0631\u0637 \u062a\u0646\u0633\u062c \u0627\u0644\u0646\u062e\u0627\u0639 \u0627\u0644\u0639\u0638\u0645\u064a \u0628\u0634\u0643\u0644 \u0643\u0628\u064a\u0631 \u0648\u062a\u0624\u062f\u064a \u0625\u0644\u0649 \u0636\u0639\u0641 \u062c\u0648\u062f\u0629 \u0627\u0644\u062d\u064a\u0627\u0629 \u0628\u0633\u0628\u0628 \u0627\u0644\u0623\u0639\u0631\u0627\u0636 \u0627\u0644\u0645\u0630\u0643\u0648\u0631\u0629 \u0623\u0639\u0644\u0627\u0647.<\/p>\n<p>\u064a\u0645\u0643\u0646 \u0623\u0646 \u062a\u0624\u062f\u064a \u0622\u0644\u064a\u0627\u062a \u0627\u0644\u0645\u0631\u0636 \u0627\u0644\u0645\u0648\u0635\u0648\u0641\u0629 \u0623\u0639\u0644\u0627\u0647 \u0625\u0644\u0649 \u0627\u0631\u062a\u0641\u0627\u0639 \u0636\u063a\u0637 \u0627\u0644\u062f\u0645 \u0648\u0627\u0631\u062a\u0641\u0627\u0639 \u0627\u0644\u0636\u063a\u0637 \u0641\u064a \u0627\u0644\u062f\u0648\u0631\u0629 \u0627\u0644\u062f\u0645\u0648\u064a\u0629 \u0627\u0644\u0631\u0626\u0648\u064a\u0629 (\u0627\u0631\u062a\u0641\u0627\u0639 \u0636\u063a\u0637 \u0627\u0644\u062f\u0645 \u0627\u0644\u0631\u0626\u0648\u064a) \u0648\u062e\u0644\u0644 \u0648\u0638\u064a\u0641\u064a \u0641\u064a \u0627\u0644\u0643\u0644\u0649\u060c \u0645\u0646 \u0628\u064a\u0646 \u0623\u0645\u0648\u0631 \u0623\u062e\u0631\u0649. \u064a\u0645\u0643\u0646 \u0623\u0646 \u062a\u0624\u062f\u064a \u0647\u0630\u0647 \u0627\u0644\u062a\u063a\u064a\u064a\u0631\u0627\u062a \u0625\u0644\u0649 \u0636\u0631\u0631 \u062f\u0627\u0626\u0645 \u0648\u062a\u062a\u0637\u0644\u0628 \u0645\u0631\u0627\u0642\u0628\u0629 \u0645\u0633\u062a\u0645\u0631\u0629.<\/p>\n<h3><span style=\"color: #f9a801;\">4.2 \u062c\u0644\u0637\u0627\u062a \u0627\u0644\u062f\u0645 (\u062a\u062c\u0644\u0637 \u0627\u0644\u062f\u0645 \/ \u0627\u0644\u0627\u0646\u0635\u0645\u0627\u0645 \u0627\u0644\u062e\u062b\u0627\u0631\u064a)<\/span><\/h3>\n<p>\u0625\u0646 \u0623\u0643\u062b\u0631 \u0627\u0644\u0645\u0636\u0627\u0639\u0641\u0627\u062a \u0627\u0644\u062a\u064a \u064a\u064f\u062e\u0634\u0649 \u062d\u062f\u0648\u062b\u0647\u0627 \u0645\u0646 \u0641\u0631\u0637 \u0641\u0631\u0637 \u062d\u0645\u0648\u0636\u0629 \u0627\u0644\u062f\u0645 \u0647\u064a \u0627\u0644\u0625\u0635\u0627\u0628\u0629 \u0628\u062c\u0644\u0637\u0627\u062a \u0627\u0644\u062f\u0645 (\u0627\u0644\u062c\u0644\u0637\u0627\u062a\/\u0627\u0644\u0627\u0646\u0635\u0645\u0627\u0645 \u0627\u0644\u062e\u062b\u0627\u0631\u064a)\u060c \u0648\u0627\u0644\u062a\u064a \u064a\u0645\u0643\u0646 \u0623\u0646 \u062a\u0624\u062f\u064a \u0625\u0644\u0649 \u0627\u0646\u0633\u062f\u0627\u062f \u0627\u0644\u0623\u0648\u0639\u064a\u0629 \u0627\u0644\u062f\u0645\u0648\u064a\u0629. 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\u0627\u0644\u0628\u064a\u0627\u0646\u064a \u0627\u0644\u062a\u0627\u0644\u064a \u0645\u0641\u0647\u0648\u0645 \u0627\u0644\u0639\u0644\u0627\u062c \u0641\u064a \u062d\u0627\u0644\u0629 \u0627\u0646\u062d\u0644\u0627\u0644 \u0627\u0644\u062f\u0645 PNH:<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-7139 size-full\" src=\"https:\/\/aa-pnh.org\/wp-content\/uploads\/PNHalgorithAR2024.png\" alt=\"\" width=\"1534\" height=\"1422\" \/><\/p>\n<p><em>\u062a\u0645\u062b\u064a\u0644 \u0645\u0628\u0633\u0637 \u0644\u062e\u0648\u0627\u0631\u0632\u0645\u064a\u0629 \u0627\u0644\u0639\u0644\u0627\u062c.<\/em> <em>\u064a\u0645\u0643\u0646 \u0627\u0644\u0627\u0637\u0644\u0627\u0639 \u0639\u0644\u0649 \u0627\u0644\u062e\u0648\u0627\u0631\u0632\u0645\u064a\u0629 \u0627\u0644\u0643\u0627\u0645\u0644\u0629 \u0641\u064a \u0625\u0631\u0634\u0627\u062f\u0627\u062a \u0645\u0646\u0638\u0645\u0629 \u0627\u0644\u0635\u062d\u0629 \u0627\u0644\u0639\u0627\u0644\u0645\u064a\u0629 \u0639\u0644\u0649 \u0645\u0648\u0642\u0639<\/em> <a href=\"https:\/\/www.onkopedia.com\/de\/onkopedia\/guidelines\/paroxysmale-naechtliche-haemoglobinurie-pnh\/@@guideline\/html\/index.html\">\u0623\u0648\u0646\u0643\u0648\u0628\u064a\u062f\u064a\u0627<\/a>.<\/p>\n<h3><span style=\"color: #f9a801;\">5.2 \u0627\u0644\u0639\u0644\u0627\u062c \u0627\u0644\u0645\u062d\u062f\u062f<\/span><\/h3>\n<p>\u0628\u0645\u0627 \u0623\u0646 <a href=\"#komplsys\">\u0627\u0644\u0646\u0638\u0627\u0645 \u0627\u0644\u062a\u0643\u0645\u064a\u0644\u064a<\/a> \u064a\u0647\u0627\u062c\u0645 \u062e\u0644\u0627\u064a\u0627 \u0627\u0644\u062f\u0645 \u0627\u0644\u062d\u0645\u0631\u0627\u0621 \u0627\u0644\u0645\u062a\u063a\u064a\u0631\u0629 \u0645\u0631\u0636\u064a\u0627\u064b\u060c \u064a\u0645\u0643\u0646 \u0643\u0628\u062d \u0627\u0644\u062a\u062f\u0645\u064a\u0631 \u0639\u0646 \u0637\u0631\u064a\u0642 \u062a\u062b\u0628\u064a\u0637 \u062c\u0632\u0621 \u0645\u0646\u0647.<\/p>\n<p>\u062a\u062a\u0645\u062b\u0644 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\u0623\u0648 \u0627\u0644\u0628\u0631\u0648\u0641\u064a\u0633\u0648\u0631 \u0627\u0644\u062f\u0643\u062a\u0648\u0631 \u0647\u0648\u0628\u0631\u062a \u0634\u0631\u064a\u0632\u0646\u0645\u0627\u064a\u0631 (<a href=\"mailto:h.schrezenmeier@blutspende.de\">h.schrezenmeier(at)blutspende.de<\/a>) \u0639\u0628\u0631 \u0627\u0644\u0628\u0631\u064a\u062f \u0627\u0644\u0625\u0644\u0643\u062a\u0631\u0648\u0646\u064a.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>\u0628\u064a\u0644\u0629 \u0627\u0644\u0647\u064a\u0645\u0648\u063a\u0644\u0648\u0628\u064a\u0646 \u0627\u0644\u0628\u0648\u0644 \u0627\u0644\u0647\u064a\u0645\u0648\u063a\u0644\u0648\u0628\u064a\u0646\u064a \u0627\u0644\u0644\u064a\u0644\u064a \u0627\u0644\u0627\u0646\u062a\u064a\u0627\u0628\u064a (PNH) 1 \u0645\u0627 \u0647\u0648 PNH 2 \u0627\u0644\u0623\u0639\u0631\u0627\u0636 3 \u0627\u0644\u062a\u0634\u062e\u064a\u0635 4 \u0627\u0644\u0645\u0633\u0627\u0631 \u0627\u0644\u0633\u0631\u064a\u0631\u064a 5 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\u0639\u0646&#8230;<\/p>\n","protected":false},"author":12,"featured_media":0,"parent":7154,"menu_order":20,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-7133","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/aa-pnh.org\/ar\/wp-json\/wp\/v2\/pages\/7133","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/aa-pnh.org\/ar\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/aa-pnh.org\/ar\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/aa-pnh.org\/ar\/wp-json\/wp\/v2\/users\/12"}],"replies":[{"embeddable":true,"href":"https:\/\/aa-pnh.org\/ar\/wp-json\/wp\/v2\/comments?post=7133"}],"version-history":[{"count":9,"href":"https:\/\/aa-pnh.org\/ar\/wp-json\/wp\/v2\/pages\/7133\/revisions"}],"predecessor-version":[{"id":8643,"href":"https:\/\/aa-pnh.org\/ar\/wp-json\/wp\/v2\/pages\/7133\/revisions\/8643"}],"up":[{"embeddable":true,"href":"https:\/\/aa-pnh.org\/ar\/wp-json\/wp\/v2\/pages\/7154"}],"wp:attachment":[{"href":"https:\/\/aa-pnh.org\/ar\/wp-json\/wp\/v2\/media?parent=7133"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}