Updated: 5/1/2020

Myelofibrosis

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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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