The pathophysiology, prevention, and treatment of stroke in sickle cell disease.
OBJECTIVE
Stroke is one of the most devastating complications of sickle cell disease, but current research has led to improved understanding of its pathogenesis and to new approaches in the prevention of both primary and secondary stroke. This review focuses on advances reported in the past 2 years.


RESULTS
New concepts in the pathophysiology of central nervous system events in sickle cell disease have centered around hemolytic anemia and nitric oxide metabolism. Genetic risk factors are now being explored. Major improvement in primary stroke prevention has occurred through transcranial Doppler ultrasonography screening, but utilization of this technique is far from optimal. Hydroxyurea is now being tested as an alternative approach to chronic transfusion for secondary stroke prevention through a multicenter trial. Other studies are addressing the management of silent infarcts and nocturnal hypoxemia.


CONCLUSIONS
Increased understanding of the etiology and pathogenesis of stroke in sickle cell disease should eventually lead to improved management of all central nervous system complications. Alternative secondary stroke prevention with hydroxyurea may allow patients to avoid dependence on life-long chronic transfusion. Primary stroke prevention through transcranial Doppler ultrasonography screening may ultimately yield a dramatic reduction in the incidence of stroke in sickle cell disease.
Wang
Winfred C
WC
Department of Hematology, St Jude Children's Research Hospital, Memphis, TN 38105, USA. winfred.wang@stjude.org
D000755:Anemia, Sickle Cell / Q000150:complications* / Q000628:therapy; D001803:Blood Transfusion; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D020521:Stroke / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy; D017585:Ultrasonography, Doppler, Transcranial
D006918:Hydroxyurea