Cerebral vasculopathy is the most severe complication affecting children with sickle cell anemia. Significant progress has been made in the management of sickle cell anemia cerebral vasculopathy, including early transcranial Doppler screening, chronic transfusion, andhydroxyurea. Nevertheless, for patients with a potential matched-sibling donor (MSD), stem cell transplantation (SCT) is now the treatment offering the best cerebral vasculopathy outcome. In the absence of MSD,alternative SCT should be recommended only in those with worsening cerebral vasculopathy despite standard treatments, and should be limited to related haplo-identical SCT undertaken in controlled studies.
Keywords: Hydroxyurea; Sickle cell anemia; Silent cerebral infarct; Stroke; Transcranial doppler; Transfusion.
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