Published online 4 December 2008
Haematologica, Vol 94, Issue 1, 123-126 doi:10.3324/haematol.13610
Copyright © 2009 by Ferrata Storti Foundation
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Sickle Cell Disease

Effect of transfusion therapy on cerebral vasculopathy in children with sickle-cell anemia

Brigitte Bader-Meunier1, Suzanne Verlhac2, Monique Elmaleh-Bergès2, Ghislaine Ithier3, Fatiha Sellami4, Sonia Faid4, Florence Missud3, Rolande Ducrocq5, Corinne Alberti6, Isabelle Zaccaria6, Andre Baruchel3, Malika Benkerrou3

1 Assistance Publique-Hôpitaux de Paris, Service d’Immuno-Hématologie-Oncologie Pédiatrique, Hôpital Necker, Paris
2 Assistance Publique-Hôpitaux de Paris, Service d’Imagerie Pédiatrique, Hôpital Robert Debré, Paris
3 Assistance Publique-Hôpitaux de Paris, Service d’Hématologie-Oncologie Pédiatrique, Hôpital Robert Debré, Paris
4 Etablissement français du sang, Ile de France, Hôpital Robert Debré, Paris
5 Assistance Publique-Hôpitaux de Paris, Service de biochimie génétique, Hôpital Robert Debré, Paris
6 Assistance Publique-Hôpitaux de Paris, Unité d’épidémiologie clinique, Hôpital Robert Debré, Paris, France; Inserm, CIE 5, Paris, France

Correspondence: Brigitte Bader-Meunier, Service d’Hématologie pédiatrique, Hôpital Necker, 149 rue de Sèvres, 75015 Paris, France. E-mail:brigitte.bader-meunier{at}nck.aphp.fr

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.

Key words: sickle-cell anemia, children, cerebral vasculopathy, stroke.