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Updated: May 1 2020


  • Introduction
    • Neoplasia of mature myeloid cells
      • most notably megakaryocytes
    • Most commonly seen in older adults (5th/6th decade)
    • Result of a JAK2 kinase mutation
    • Megakaryocyte hyperplasia results in fibrotic obliteration of the bone marrow
      • result of increased production of PDGF
  • Presentation
    • Symptoms
      • splenomegaly
        • secondary to extramedullary hematopoiesis
      • bleeding
        • megakaryocytes are dysfunctional
      • thrombosis
        • platelets can rupture which stimulates coagulation cascade
  • Evaluation
    • Peripheral blood smear
      • tear-drop RBC
        • membrane is disrupted when RBC passed through fibrosis to leave bone marrow
      • nucleated RBCs
      • band granulocytes
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