Hematology and Oncology Book

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Cerebrovascular Accident in Sickle Cell Anemia

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  1. See Also
    1. Sickle Cell Anemia
  2. Epidemiology: Incidence
    1. Children: 5% (silent CVA may occur in up to 17%)
    2. Lifetime risk: 25%
  3. Pathophysiology
    1. Blockage of Internal Carotid Artery
  4. Symptoms: Findings suggestive of acute CVA
    1. Behavior change
    2. Persistent Headache
  5. Imaging
    1. Transcranial doppler ultrasound
      1. Routine screening starts at age 2 years
      2. Evaluate transcranial blood flow velocity (TBV)
        1. High CVA risk suggested by TBV >200 cm/sec
    2. MRI Head or CT Head
      1. Indicated for suspected acute cerebrovascular event
  6. Management: Exchange Transfusions
    1. Reduce proportion of Hemoglobin S <50%
    2. Maintain this level through childhood
  7. References
    1. Adams (1998) N Engl J Med 339:5

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