{"id":5347,"date":"2021-05-15T23:53:42","date_gmt":"2021-05-15T21:53:42","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/hematological-oncology\/myelofibrosis\/"},"modified":"2021-05-24T15:18:18","modified_gmt":"2021-05-24T13:18:18","slug":"myelofibrosis","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/myeloproliferative-disorders\/myelofibrosis\/","title":{"rendered":"Myelofibrosis"},"content":{"rendered":"<span class=\"block-heading\" id=\"header_1\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Background<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<p class=\"wp-block-paragraph\">Myeloproliferative diseases are neoplastic diseases of the myeloid hematopoietic cells in the bone marrow.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>One or more groups of cells proliferate uncontrollably, releasing mature but defective cells into the circulation; the erythropoiesis becomes ineffective, leading to extramedullary erythropoiesis; hepatosplenomegaly is common.<\/li><li>The 3 main non-leukemic disorders in this group are:<ul><li>Polycythemia vera<\/li><li>Essential thrombocythemia<\/li><li>Myelofibrosis<\/li><\/ul><\/li><li>The &#8220;driver-mutation&#8221; is most often a mutation of the <strong>tyrosine kinase proteins<\/strong> (JAK2).<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Myelofibrosis<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<p class=\"wp-block-paragraph\"><strong>Decreased hematopoiesis<\/strong>&nbsp;due to&nbsp;<strong>fibrosis of the bone marrow<\/strong>&nbsp;(hence the name).<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li><strong>Myelo<\/strong> = of the bone marrow; <strong>fibrosis<\/strong> = replacement of the tissue with collagen.<\/li><li>Most probably,&nbsp;<strong>megakaryocytes&nbsp;<\/strong>releasing PDGF and TGF-\u03b2, activating fibroblasts<ul><li>Resulting in increased ECM deposition = fibrosis.<\/li><\/ul><\/li><li>Most cases present with <strong>JAK2<\/strong> (most commonly), <strong>CALR<\/strong>, or <strong>MPL<\/strong> mutations.<\/li><li>Consists of 2 stages:<ul><li>Hypercellular stage<ul><li>Cellular proliferation due to the mutation<\/li><\/ul><\/li><li>Hypocellular stage<ul><li>Due to bone marrow fibrosis<\/li><\/ul><\/li><\/ul><\/li><li>Fibrosis will lead to<strong>&nbsp;extramedullary hematopoiesis<\/strong><ul><li>Liver<\/li><li>Spleen<\/li><\/ul><\/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 title2\">Clinical presentation<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<ul class=\"wp-block-list\"><li>Anemia and bleeding disorders<\/li><li>Massive hepatosplenomegaly leading to abdominal pain (the main physical finding)<\/li><li>Night sweats, weightloss, fever<\/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 title2\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<ul class=\"wp-block-list\"><li>CBC (anemia; late diseases shows thrombocytopenia and leukopenia)<\/li><li>Chemistry (elevated LDH and urate)<\/li><li>Bone marrow sample (hypocellular with extensive fibrosis)<\/li><li>Genetic analysis (FISH; displaying JAK2 mutations)<\/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 title2\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<ul class=\"wp-block-list\"><li>Supportive:<ul><li>Blood transfusion<\/li><li>Splenectomy<\/li><\/ul><\/li><li>Specific:<ul><li>Ruxolitinib (JAK2 inhibitor)<\/li><li>Hydroxycarbamide<\/li><li>Thalidomide and lenalidamide<\/li><\/ul><\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Background<\/h3><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Myelofibrosis<\/h3><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>Background Myeloproliferative diseases are neoplastic diseases of the myeloid hematopoietic cells in the bone marrow. One or more groups of cells proliferate uncontrollably, releasing mature but defective cells into the circulation; the erythropoiesis becomes ineffective, leading to extramedullary erythropoiesis; hepatosplenomegaly is common. The 3 main non-leukemic disorders in this group are: Polycythemia vera Essential thrombocythemia [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5476,"menu_order":2,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5347","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>Myelofibrosis &#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\/oncological-disorders\/myeloproliferative-disorders\/myelofibrosis\/\" \/>\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\\\/oncological-disorders\\\/myeloproliferative-disorders\\\/myelofibrosis\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/oncological-disorders\\\/myeloproliferative-disorders\\\/myelofibrosis\\\/\",\"name\":\"Myelofibrosis &#8211; 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