What does ASH recommend for the prevention of stroke in patients with sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Primary stroke prevention in children:

  • Annual transcranial Doppler (TCD) screening for children aged 2-16 years with hemoglobin SS (HbSS) or HbSβ0 thalassemia (all income settings)
  • Regular blood transfusions for a minimum of 1 year for children aged 2-16 years with HbSS or HbSβ0 thalassemia who have abnormal TCD velocities and live in a high-income setting; this refers to a setting that permits regular blood transfusion therapy, typically every 3-4 weeks, to maintain the maximum HbS level below 30% and the hemoglobin level above 9.0 g/dL, to reduce stroke risk.

Management of suspected or confirmed ischemic stroke or transient ischemic attack (all income settings):

Prompt blood transfusion is recommended for children or adults with SCD who have acute neurologic deficits, including transient ischemic attack (TIA). The transfusion should not be delayed beyond 2 hours of acute neurologic symptom presentation. Individual patient factors and local transfusion resources determine the type of transfusion provided (simple, modified exchange, or apheresis).

Secondary prevention of ischemic strokes:

  • For children with HbSS or HbSβ 0 thalassemia and a history of prior ischemic stroke, blood transfusion goals for secondary stroke prevention are to increase the hemoglobin level above 9 g/dL at all times and maintain the HbS level at < 30% of total hemoglobin until the time of the next transfusion.

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