[Stroke prevention in sickle-cell disease: results, hurdles and future perspectives].
Sickle cell disease (SCD) is the most frequent cause of stroke during infancy, and stroke is the most serious complication of SCD in children. Sludge-induced distal vasculopathy explains 25% of strokes in SCD, while proximal vasculopathy is responsible for 75% of cases. The stenoses observed in SCD-related proximal vasculopathy are progressive and can be detected by transcranial Doppler (TCD), a reliable, non-invasive and low-cost imaging method. High velocities (> 2 mls) are associated with a 40% risk of stroke within 36 months, but initiation of a transfusion program maintaining the HbS level under 30% reduces the risk to less than 2%. TCD must be performed in all children at 12-18 months of age to detect cerebral vasculopathy and prevent stroke. This approach has been adopted in our institution, based on a cohort of SCD newborns screened at birth: the risk of stroke was reduced from the expected 11% to less than 2% at 18 years. Genoidentical stem cell transplantation, which safely obviates the need for transfusion programs and provides a 95% chance of cure, should be offered early to patients at risk of stroke. When possible, sibling cord blood cryopreservation is recommended
Bernaudin
Françoise
F
Hématologie-Pédiatrie, Hôpital Intercommunal de Créteil, 40 avenue de Verdun 94000 Créteil.
Verlhac
Suzanne
S
D000755:Anemia, Sickle Cell / Q000150:complications*; D002561:Cerebrovascular Disorders / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D002648:Child; D005544:Forecasting; D006801:Humans; D008279:Magnetic Resonance Imaging; D020521:Stroke / Q000000981:diagnostic imaging / Q000517:prevention & control*; D017585:Ultrasonography, Doppler, Transcranial