{"id":5345,"date":"2021-05-15T23:53:42","date_gmt":"2021-05-15T21:53:42","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/hematological-oncology\/essential-thrombocythemia\/"},"modified":"2022-02-12T11:42:11","modified_gmt":"2022-02-12T09:42:11","slug":"essential-thrombocythemia","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/myeloproliferative-disorders\/essential-thrombocythemia\/","title":{"rendered":"Essential thrombocythemia"},"content":{"rendered":"<span class=\"block-heading\" id=\"header_1\">\n<h3 class=\"wp-block-heading\" id=\"Background\" class=\"wp-block-heading\" id=\"Background\" 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 \u201cdriver-mutation\u201d is most often a mutation of the&nbsp;<strong>tyrosine kinase proteins<\/strong>&nbsp;(JAK2).<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h3 class=\"wp-block-heading\" id=\"essential-thrombocythemia\" class=\"wp-block-heading\" id=\"essential-thrombocythemia\" class=\"title_collection title1\">Essential thrombocythemia<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<p class=\"wp-block-paragraph\">Essential thrombocythemia is a clonal proliferation of megakaryocytes leading to an overproduction of platelets.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>The produced platelets are defective.<\/li><li>Most cases present with <strong>JAK2<\/strong> (most commonly), <strong>CALR<\/strong> (best prognosis), or <strong>MPL<\/strong> (rare) mutations.<\/li><li>May progress to myelofibrosis.<\/li><li>Rarely, patients with extremely high platelet count (>1 million) may develop bleeding. <\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h4 class=\"wp-block-heading\" id=\"clinical-manifestation\" class=\"wp-block-heading\" id=\"clinical-manifestation\" class=\"title_collection title2\">Clinical manifestation<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<ul class=\"wp-block-list\"><li>Most cases are asymptomatic<\/li><li>Thromboses; Budd-Chiari syndrome<\/li><li>Splenomegaly<\/li><li>Rarely, spontaneous bleeding can be seen in patients with platelet count over 1T\/L.<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" id=\"diagnosis\" class=\"wp-block-heading\" id=\"diagnosis\" 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 (platelet counts &gt;450G\/L)<\/li><li>Bone marrow sample (hypercellular; megakaryocyte proliferation with defective morphology)<\/li><li>Genetic tests (demonstrating JAK2\/CALR\/MPL mutations)<\/li><li>Platelet function tests (abnormal)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_5\">\n<h4 class=\"wp-block-heading\" id=\"treatment\" class=\"wp-block-heading\" id=\"treatment\" class=\"title_collection title2\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<p class=\"wp-block-paragraph\">The purpose is to reduce the risk for thrombotic events.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Aspirin 75mg\/day<\/li><li>If platelet count &gt;1500G\/L, use hydroxycarbamide<\/li><li>Interferon-alpha<\/li><li>Ruxolitinib (JAK2 inhibitor)<\/li><li>Anagrelide&nbsp;(prevents platelet maturation)<\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h3 class=\"wp-block-heading\" id=\"Background\" class=\"wp-block-heading\" id=\"Background\">Background<\/h3><h3 class=\"wp-block-heading\" id=\"essential-thrombocythemia\" class=\"wp-block-heading\" id=\"essential-thrombocythemia\">Essential thrombocythemia<\/h3><h4 class=\"wp-block-heading\" id=\"clinical-manifestation\" class=\"wp-block-heading\" id=\"clinical-manifestation\">Clinical manifestation<\/h4><h4 class=\"wp-block-heading\" id=\"diagnosis\" class=\"wp-block-heading\" id=\"diagnosis\">Diagnosis<\/h4><h4 class=\"wp-block-heading\" id=\"treatment\" class=\"wp-block-heading\" id=\"treatment\">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":1,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5345","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>Essential thrombocythemia &#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\/essential-thrombocythemia\/\" \/>\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\\\/essential-thrombocythemia\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/oncological-disorders\\\/myeloproliferative-disorders\\\/essential-thrombocythemia\\\/\",\"name\":\"Essential thrombocythemia &#8211; 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