Polycythemia Vera

• Polycythemia vera 


  A myeloproliferative disorder associated with a Janus kinase-2 (JAK2) mutation causing neoplastic proliferation of the hematopoietic progenitor cells
• Polycythemia Vera

  Is characterized by hyperplasia of the bone marrow.
• Polycythemia Vera

  Whenever the bone marrow is affected, all the blood cells are affected.

  • RBC increase = erythrocytosis
  • WBC increase = leukocytosis
  • Platelets increase = thrombocytosis
• Polycythemia Vera: Signs and Symptoms

  Erythrocytosis - high RBC

  • "Ruddy" complexion
  • Poor circulation - due to increased blood viscosity causing headache, visual changes, chest pain, orthopnea
  • Gout - increased uric acid
  • Splenomegaly - enlarged organ

  Leukocytosis - high WBC

  • Pruritus - due to increased histamine from high WBC

  Thrombocytosis - high Platelets

  • Increased risk for thromboembolism
• Polycythemia Vera: Management

  1. Increased fluid intake. To reduce blood viscosity and prevent thromboembolism
  2. Administer antihistamine to relieve pruritus
  3. Administer Allopurinol to decrease uric acid.

  Patient teaching: Avoid high altitude. There is low oxygen concentration and this triggers the kidneys to secrete erythropoietin. This in turn, stimulates increased production of RBC (e.g., climbing mountains, airplane travel)