Polycythemia vera

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Background

  • Chronic myeloproliferative disorder
  • Abnormal proliferation is seen in all 3 cell lines
  • Typically in elderly
  • Can be asymptomatic or symptomatic

Clinical Presentation

  • Severe burning pain in the hands or feet accompanied by a reddish or bluish coloration of the skin
  • Suspected with any combination of the following: [1]
    • Abnormally elevated hemoglobin levels (>18 g/dL in men; 16 g/dL in women)
    • Normal oxygen saturation
    • Bleeding complications
    • Portal vein thrombosis
    • Splenomegaly
    • Plethora
    • Pruritus after bathing

DDx

  • Essential thrombocytosis (ET)
  • Chronic myelogenous leukemia (CML)
  • Leukemoid reactions
  • Hypoxia

Labs

  • CBC
  • Chem 7
  • ESR
  • EPO
  • Bone Marrow Bx sometimes needed

Treatment

  • Phlebotomy
  • ASA 81 mg
  • Myelosuppressive agents

Also See

Sources

http://emedicine.medscape.com/article/205114-differential