Polycythemia vera

Background

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

Clinical Features

  • 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

Differential Diagnosis

Evaluation

Workup

  • CBC
  • Chem 7
  • ESR
  • EPO decreased
  • Bone marrow biopsy sometimes needed

Evaluation[2]

  • Criteria 1: All category A
  • Criteria 2: First 3 of category A and any 2 of category B
Category A Category B
Increased RBC Mass (Men >18.5, Women >16.5) Thrombocytosis (>400,000)
Normal SPO2 (>92%) Leukocytosis (>12,000)
Splenomegaly Leukocyte Alk Phos >100
Vit B12 >900

Management

  • Phlebotomy
  • ASA 81mg
  • Myelosuppressive agents (hydroxyurea, interferon alpha)

Also See

References

  1. http://emedicine.medscape.com/article/205114-differential
  2. Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,
Last modified on 5 October 2016, at 05:16