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Myeloproliferative Neoplasms: Learning Objectives

Medical Importance

The chronic myeloproliferative diseases are clonal hematopoietic stem cell disorders in which the progenitor cells of one or more of the hematopoietic lineages proliferate excessively. In contrast to the situation in myelodysplastic syndromes and acute leukemia (in which the hematopoietic precursor cells do not undergo terminal differentiation), cellular differentiation from immature precursor cells to mature blood cells proceeds in a normal manner. Consequently, mature leukocytes, red cells, and platelets are overproduced in patients with these diseases and generally at least one of the blood cell counts is high. Some patients with myeloproliferative neoplasms can present with seemingly trivial symptoms, and in others the diagnoses is made during or after a life-threatening complication. Symptoms can include itching, burning feet, bleeding, or thrombosis. Effective treatment can minimize symptoms and prevent major debilitating complications, but these diseases are incurable unless the neoplastic clone is destroyed and replaced by normal hematopoietic stem cells.

Objectives

  1. Name the four major myeloproliferative neoplasmss and describe the pathophysiologic features shared by these disorders.
  2. Name four causes of reactive or secondary thrombocytosis.
  3. Name the two major complications of essential thrombocythemia.
  4. Describe the typical physical exam, and blood and bone marrow findings in patients with chronic idiopathic myelofibrosis.
  5. Describe the common mutation associated with polycythemia vera and its biological consequences.
  6. Describe the clinical features and complications of polycythemia vera.
  7. Describe treatment approaches to polycythemia vera.
  8. Name three causes of splenomegaly.