polycythemia vera

  • erythrocytosis
  • questions to ask
    • smoking history
    • hormonal therapy (testosterone increases Hgb)
    • OSA hx
    • renal/liver cancer
    • burning pain in hands/feet
    • itching
    • fatigue
  • physical exam findings
    • facial plethora
    • red extremities
    • clubbing
    • splenomegaly
  • labs
    • iron profile
    • LDL
    • uric acid
    • EPO
    • JAK2: both mutations
    • can have both leukocytosis and thrombocytosis

Diagnosis

  • initial bone marrow baseline
    • eventually 1/5 will progress to myelofibrosis

Treatment

  • patients on testosterone therapy require hematocrit monitoring; testosterone supplementation should be decreased/discontinued if hematocrit levels are >54%.
  • decrease thrombosis
  • prevent bleed
  • decrease sx
  • no known agents to decrease progression to AML/MDS/MF
  • low risk
    • < 60
    • no hx of thrombosis
  • high risk

    • 60

    • hx of thrombosis
  • Ruxolitinib

Prognosis

  • untreated: 18 months
  • treated: 13 years