polycythemia vera

• related: Hemeonc, essential thrombocytosis

• 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