Polycythemia Vera (P Vera)

Primary erthrocytosis

Etiology

  • Proliferative disorder where bone marrow inappropriately produces RBCs, non-EPO dependent
  • Associated w/JAK2 mutation
  • Secondary erythrocytosis is caused by elevated EPO from tumors (e.g. RCC, HCC, fibroids) chronic hypoxemia (e.g. OSA, severe COPD, other), meds (e.g. testosterone). These processes don't lead to hypercoaguable states.

Risk Factors

  • Age 60-65

Symptoms

  • Asx until hgb significantly elevated
  • Itching exacerbated by hot shower
  • Sx related to sludging→ vague abd pain, sob, wt loss, head ache, TIA, DVT, stroke
  • Higher risk for arterial events (in particular if no other RFs)
  • Higher risk for DVTs, consider in particular if unusual locations (mensenteric, cerebral veins, etc) and/or no other RFs

Physical Exam Findings

  • Splenomegaly
  • Ruddy complexion

Tests