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Polycythemia Vera

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Polycythemia Vera

  1. 1. Polycythemia Vera Patrick Carter MPAS, PA-C Clinical Medicine 1 April 4, 2011
  2. 2. Polycythemia Vera <ul><li>Increased red blood cell mass </li></ul><ul><li>Acquired myeloproliferative disorder </li></ul><ul><ul><li>Overproduction of all 3 cell lines, mostly RBCs </li></ul></ul><ul><ul><li>Increased HCT >54/51 (male/female) </li></ul></ul><ul><li>Results from a mutation in JAK2, a signaling molecule in 95% of cases </li></ul><ul><li>Occurrence 2-18 per 100,00 (increases with age) </li></ul><ul><li>Rarely occurs in persons under 40 years old </li></ul>
  3. 3. Polycythemia Vera <ul><li>Signs and symptoms </li></ul><ul><ul><li>Symptoms related to expanded blood volume and increased viscosity </li></ul></ul><ul><ul><ul><li>Headache, tinnitus, dizziness, blurred vision, TIAs, and fatigue </li></ul></ul></ul><ul><ul><li>Generalized pruritus (aquagenic) </li></ul></ul><ul><ul><li>Engorged retinal veins </li></ul></ul><ul><ul><li>Palpable spleen (75%) </li></ul></ul><ul><ul><li>Thrombosis </li></ul></ul><ul><ul><li>Easy bleeding/bruising </li></ul></ul>
  4. 4. Polycythemia Vera <ul><li>Laboratory findings </li></ul><ul><ul><li>Elevated Hct – often >60% </li></ul></ul><ul><ul><li>Increased WBCs and platelets </li></ul></ul><ul><ul><li>Serum EPO levels are low </li></ul></ul><ul><ul><li>Decreased iron stores (Ferritin) </li></ul></ul><ul><ul><li>Increased Vitamin B12 </li></ul></ul><ul><ul><li>Increased uric acid </li></ul></ul><ul><ul><li>Presence of the JAK2 mutation </li></ul></ul><ul><ul><li>Hypercellular bone marrow </li></ul></ul>
  5. 5. Polycythemia Vera <ul><li>Treatment </li></ul><ul><ul><li>Treatment of choice is phlebotomy </li></ul></ul><ul><ul><ul><li>One unit of blood removed weekly until Hct is < 45% - then maintain Hct <45% with occasional phlebotomy </li></ul></ul></ul><ul><ul><li>Low iron diet </li></ul></ul><ul><ul><li>Myelosuppressive therapy (hydroxyurea) if </li></ul></ul><ul><ul><ul><li>High phlebotomy requirement </li></ul></ul></ul><ul><ul><ul><li>Thrombocytosis (goal to get PLTs <600,000) </li></ul></ul></ul><ul><ul><ul><li>Intractable pruritus </li></ul></ul></ul><ul><li>Prognosis </li></ul><ul><ul><li>Median survival 11-15 years </li></ul></ul><ul><ul><li>Major cause of morbidity/mortality is arterial thrombosis </li></ul></ul>

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