This retrospective study assessed the long-term effect of transfusional exchange therapy on MRA/
MRI abnormalities in 24
homozygous sickle-cell anemia (HbSS) children presenting with abnormal
brain MRA. The
median time elapsed from baseline to last available MRA was 29 months. Follow-up MRAs showed improvement, stabilization or worsening of
cerebrovascular lesions in 11, 6 and 7 patients respectively. Complete normalization of MRA was observed in 6 patients within a
mean time of 1.4 years, but
stenosis recurred at the same location in the 4 patients in whom
transfusion therapy was discontinued. Baseline severe
stenosis/occlusion of large cerebral
arteries and occurrence of
moyamoya syndrome were significantly associated with an absence of improvement of the cerebral vasculopathy. These data emphasize the
heterogeneity of the course of cerebrovasculopathy in SS children receiving chronic
transfusion. Further studies are needed to determine whether different therapeutic approaches have to be considered according to these different evolutive patterns in SS children.
