{"id":5615,"date":"2021-05-21T01:56:46","date_gmt":"2021-05-20T23:56:46","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/thrombocytopenia-and-platelet-disorders\/hemophilia-a-and-b\/"},"modified":"2021-05-26T14:29:07","modified_gmt":"2021-05-26T12:29:07","slug":"hemophilia-a-and-b","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/thrombocytopenia-and-platelet-disorders\/hemophilia-a-and-b\/","title":{"rendered":"Hemophilia A and B"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\"><strong>Hemophilia A<\/strong> (factor VIII deficiency), <strong>hemophilia B <\/strong>(Christmas disease, factor IX deficiency). Both disorders will result in bleeding and hemorrhage.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Hemophilia A is much more common than B.<\/li><\/ul>\n\n\n<span class=\"block-heading\" id=\"header_1\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Etiology<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<p class=\"wp-block-paragraph\">Both disorders are X-linked defects in genes coding for clotting factors, leading to their deficiencies.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Factor VIII is a cofactor for factor IX of the intrinsic pathway produced by endothelial cells.<\/li><li>Factor IX is a participant of the intrinsic pathway, requiring factor VIII for its activity. Its synthesis requires vitamin K.<\/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\">Classification<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<ul class=\"wp-block-list\"><li>Mild (rare bleeding)<\/li><li>Moderate (spontaneous bleeding can occur)<\/li><li>Severe (frequent bleeding)<\/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\">Clinical manifestation<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<ul class=\"wp-block-list\"><li>Bleeding during delivery<\/li><li>Post-circumcision<\/li><li>Joint (hemarthrosis) and muscle bleedings<\/li><li>Prolonged bleeding after dental treatments<\/li><li>Spontaneous hematuria and GI bleedings<\/li><li>Hemophilic pseudotumors (large encapsulated hematomas)<\/li><li>Life-threatning bleedings:<ul><li>CNS<\/li><li>Neck<\/li><li>GI<\/li><li>Retroperitona<\/li><\/ul><\/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\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<ul class=\"wp-block-list\"><li>CBC (normal)<\/li><li>PT (normal)<\/li><li>aPTT (prolonged)<\/li><li>TT (normal)<\/li><li>Bleeding time (usually normal)<\/li><li>Factor VIII clotting assay.<\/li><li>Platelet function analysis\u2010100 (PFA\u2010100) is normal<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_5\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<ul class=\"wp-block-list\"><li>In acute bleeding, immediate supplementation of FVIII &amp; FIX, plasma derived or recombinant.<ul><li>1 IU\/kg of FVIII raises factor level by 2%, administered x2 daily<\/li><li>1 IU\/kg of FIX raises factor level by 1%, administered x1 daily<\/li><\/ul><\/li><li>Prophylaxis<ul><li> FVIII: 20-40IU\/\/kg, x3 per week<\/li><li>FIX: 20-40IU\/kg, x2 per week<\/li><\/ul><\/li><li>New treatment options<ul><li>Pegylated factors (increasing their half-life)<\/li><li>Emicizumab (mAb mimicking FVIII)<\/li><li>Gene therapy <\/li><\/ul><\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_6\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Complications<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_6\">\n\n\n<ul class=\"wp-block-list\"><li>Transmission of pathogens (HIV, hepatitis)<\/li><li>Tolerance (development of antibodies)<\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Etiology<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Classification<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Clinical manifestation<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Diagnosis<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Treatment<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Complications<\/h4><\/div>","protected":false},"excerpt":{"rendered":"<p>Hemophilia A (factor VIII deficiency), hemophilia B (Christmas disease, factor IX deficiency). Both disorders will result in bleeding and hemorrhage. Hemophilia A is much more common than B. Etiology Both disorders are X-linked defects in genes coding for clotting factors, leading to their deficiencies. Factor VIII is a cofactor for factor IX of the intrinsic [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5575,"menu_order":5,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5615","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>Hemophilia A and B &#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\/thrombocytopenia-and-platelet-disorders\/hemophilia-a-and-b\/\" \/>\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\\\/thrombocytopenia-and-platelet-disorders\\\/hemophilia-a-and-b\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/thrombocytopenia-and-platelet-disorders\\\/hemophilia-a-and-b\\\/\",\"name\":\"Hemophilia A and B &#8211; 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