Myeloid metaplasia with myelofibrosis

on 7.2.09 with 0 comments

  • Proliferation of neoplastic myeloid stem cells mostly in the spleen (myeloid metaplasia)

  • Without a pre-existing syndrome (such as p. vera or CML) this is called agnogenic (idiopathic) myeloid metaplasia

  • Mostly in people > 50 YOA who c/s of splenomegaly and progressive anemia

  • Peripheral smear shows tear-shaped erythrocytes (dacrocytes), leukoerythroblastosis (from extramedullary hematopoiesis and fibrosis pushing out precursors), and abnormal (“giant”) platelets

  • Marrow typically hypocellular with atypical appearing megakaryocytes and a variable degree of fibrosis (fibroplastic proliferation from elaboration of growth factors by neoplastic megakaryocytes)

  • Myelofibrotic pattern may be seen in the “burnt out” phases of CML and p. vera

  • Course is highly variable and people often die from infection, thrombosis, bleeding, or transformation into leukemia (5-20% of cases)

  • Median survival from 1-5 years

Category: Medical Subject Notes , Pathology Notes



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