{"id":5343,"date":"2021-05-15T23:53:42","date_gmt":"2021-05-15T21:53:42","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/hematological-oncology\/polycythemia-vera\/"},"modified":"2021-05-27T13:36:16","modified_gmt":"2021-05-27T11:36:16","slug":"polycythemia-vera","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/myeloproliferative-disorders\/polycythemia-vera\/","title":{"rendered":"Polycythemia vera"},"content":{"rendered":"\n

Background<\/h3>\n<\/span>\n\n\n

Myeloproliferative diseases are neoplastic diseases of the myeloid hematopoietic cells in the bone marrow.<\/p>\n\n\n\n

  • 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>
  • The 3 main non-leukemic disorders in this group are:
    • Polycythemia vera<\/li>
    • Essential thrombocythemia<\/li>
    • Myelofibrosis<\/li><\/ul><\/li>
    • The \u201cdriver-mutation\u201d is most often a mutation of the\u00a0tyrosine kinase proteins<\/strong>\u00a0(JAK2).
      • For PV, JAK2 mutation appears in the vast majority of cases.<\/li>
      • For ET and MF, other mutations include CALR (best prognosis) and MPL (rarest).<\/li><\/ul><\/li>
      • Treatments often overlap:
        • JAK2 inhibitor (ruxolitinib)<\/li>
        • Hydroxycarbamide (hydroxyurea)<\/li>
        • INF-alpha<\/li><\/ul><\/li><\/ul>\n\n\n<\/span>\n

          Polycythemia vera<\/h3>\n<\/span>\n\n\n

          A myeloproliferative clonal proliferation resulting in the production of (mainly) RBCs.<\/p>\n\n\n\n

          • Polycythemia refers to the increase in Hgb above the normal range; polycythemia vera is a subtype of absolute polycythemia<\/strong> in which the total mass of RBCs is increased.<\/li>
          • Typically affects older adults.<\/li>
          • Most cases present with a JAK2 mutation.<\/li><\/ul>\n\n\n<\/span>\n

            Clinical presentation<\/h4>\n<\/span>\n\n\n
            • General symptoms (headaches, dyspnea, blurred vision)<\/li>
            • Redness of the skin<\/li>
            • Hemorrhages or thrombosis due to defective platelet function<\/li>
            • Splenomegaly<\/li>
            • Gout<\/li><\/ul>\n\n\n<\/span>\n

              Diagnosis<\/h4>\n<\/span>\n\n\n
              • CBC (RBC counts are elevated; granulocyte and platelet counts are also elevated; >50% hematocrit, Hgb>185g\/L in males and >165g\/L in females)<\/li>
              • Erythropoietin (low)<\/li>
              • Chemistry (elevated urea and LDH)<\/li>
              • Blood smear<\/li>
              • Bone marrow sample (hypercellular)<\/li>
              • Genetic tests (demonstrating JAK2 mutations)<\/li><\/ul>\n\n\n<\/span>\n

                Treatment<\/h4>\n<\/span>\n\n\n
                • Venesection and hydroxycarbamide to reduce the hematocrit<\/li>
                • JAK inhibitors (ruxolitinib)<\/li>
                • Alpha-interferon<\/li><\/ul>\n<\/span>

                  Background<\/h3>

                  Polycythemia vera<\/h3>

                  Clinical presentation<\/h4>

                  Diagnosis<\/h4>

                  Treatment<\/h4><\/div>","protected":false},"excerpt":{"rendered":"

                  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 […]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5476,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5343","page","type-page","status-publish","hentry"],"yoast_head":"\nPolycythemia vera – 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\/polycythemia-vera\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" 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