{"id":5268,"date":"2021-05-13T19:35:38","date_gmt":"2021-05-13T17:35:38","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/anemias\/hemolytic-anemias\/paroxysmal-nocturnal-hemoglobinuria\/"},"modified":"2021-05-22T21:44:30","modified_gmt":"2021-05-22T19:44:30","slug":"paroxysmal-nocturnal-hemoglobinuria","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/anemias\/hemolytic-anemias\/paroxysmal-nocturnal-hemoglobinuria\/","title":{"rendered":"Paroxysmal nocturnal hemoglobinuria"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\">Paroxysmal nocturnal hemoglobinuria is an acquired clonal cell disorder leading to intra- and extravascular hemolysis.<\/p>\n\n\n<span class=\"block-heading\" id=\"header_1\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Pathophysiology<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<p class=\"wp-block-paragraph\">Paroxysmal nocturnal hemoglobinuria\u00a0is an acquired mutation in the PIGA (phosphatidylinositol glycan) gene which is required for the synthesis of GPI links or &#8220;PIG tails&#8221;. GPI links attach surface markers (<strong>CD55 <\/strong>and <strong>CD59<\/strong>) that help the RBCs avoid being attacked by the complement system.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>When there are no PIG tails on the surface of RBCs, the complement system is able to attach to the RBCs and create a MAC which results in the lysis of the cell.<\/li><li>It typically happens at night as the pH of the blood decreases (the complement system is more active in acidic pH).<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Clinical presentation<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<ul class=\"wp-block-list\"><li>Hemosiderinuria leading to iron deficiency<\/li><li>Venous thrombosis<\/li><li>Arterial thrombosis (less frequent)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<ul class=\"wp-block-list\"><li>Flow cytometry (CD55, CD59)<\/li><li>Ham test (demonstration of RBC lysis at low pH) or by adding sucrose<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<ul class=\"wp-block-list\"><li><strong>Eculizumab<\/strong>, an anti-C5 monoclonal antibody<\/li><li>LMWH for the thrombosis<\/li><li>Steroids<\/li><li>Bone marrow transplant<\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Pathophysiology<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Clinical presentation<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Diagnosis<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Treatment<\/h4><\/div>","protected":false},"excerpt":{"rendered":"<p>Paroxysmal nocturnal hemoglobinuria is an acquired clonal cell disorder leading to intra- and extravascular hemolysis. Pathophysiology Paroxysmal nocturnal hemoglobinuria\u00a0is an acquired mutation in the PIGA (phosphatidylinositol glycan) gene which is required for the synthesis of GPI links or &#8220;PIG tails&#8221;. GPI links attach surface markers (CD55 and CD59) that help the RBCs avoid being attacked [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5264,"menu_order":2,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5268","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.9 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Paroxysmal nocturnal hemoglobinuria &#8211; Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/anemias\/hemolytic-anemias\/paroxysmal-nocturnal-hemoglobinuria\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/anemias\\\/hemolytic-anemias\\\/paroxysmal-nocturnal-hemoglobinuria\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/anemias\\\/hemolytic-anemias\\\/paroxysmal-nocturnal-hemoglobinuria\\\/\",\"name\":\"Paroxysmal nocturnal hemoglobinuria &#8211; 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