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Essential Thrombocythemia

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Essential Thrombocythemia

  • See Also
  1. Thrombocytosis
  • Epidemiology
  1. More common in women
  • Symptoms and signs
  1. Common
    1. Headache
    2. Paresthesias
  2. Less common
    1. Thrombotic events
    2. Bleeding events
  • Differential Diagnosis
  1. Reactive Thrombocytosis
  2. Clonal Thrombocytosis
  3. Chronic myeloproliferative disorder
  • Labs (rule-out differential diagnosis above)
  1. C-Reactive Protein (CRP) Normal
  2. Erythrocyte Sedimentation Rate Normal
  3. Fibrinogen Level Normal
  4. Bone Marrow Biopsy Normal
  • Management
  1. Age Under 60 years
    1. Low risk patients
      1. Consider low dose Aspirin
    2. Intermediate risk patients
      1. Platelet Count <1500 x10^3/uL
        1. Anagrelide
        2. Low dose Aspirin
      2. Platelet Count >1500 x10^3/uL
        1. Anagrelide
    3. High risk patients
      1. Hydroxyurea
      2. Anagrelide
      3. Low dose Aspirin
  2. High risk patients over age 60 years
    1. Low dose Aspirin
    2. Hydroxyurea
  3. Women of child bearing age
    1. Consider low dose Aspirin
    2. Interferon alfa in high risk patients
  • Complications (low Incidence)
  1. Acute Leukemia
  2. Myelofibrosis
  • Prognosis
  • Factors predictive of poor prognosis
  1. Age over 60 years
  2. Thrombosis history
  • References
  1. Tefferi (2001) Am J Med 109:146 [PubMed]