Cerebrovascular accidents in sickle cell disease. Risk factors and blood transfusion influence

This study presents a series of 34 sickle cell patients with one or more cerebrovascular accidents (CVA). Risk factors were studied in a subgroup of 19 patients whose clinical and biological characteristics were compared to those of a group of 444 sickle cell patients without CVA. The only risk factor discovered was a past history of purulent meningitis, which was significantly more frequent in sickle cell patients than in those without CVA (P<0.0001). No biological or radiological factor affecting the risk of recurrence was found. The risk of recurrence, neurological defects or death after subsequent CVA justify long-term transfusion treatment in patients presenting with a second CVA. However our study shows that 10 patients who were not transfused after their first CVA had no recurrences, (median follow up = 7.9 years; 2–18 years), providing a basis for discussion on the indications of long-term transfusion therapy for sickle cell patients presenting with their first CVA.D. Bachir (Creteil), J. Badoual (Paris), H. Bataille (Pointe-A-Pitre), P. Beauvais (Paris), P. Begue (Paris), M. Belloy (Paris), E. Vilmer (Paris), J.J. Benichou (Kremlin Bicetre), F. Boccara (Paris), T. Cynober (Kremlin Bicetre), J. Elion (Paris), J. Feingold (Paris), M. Guilloud-Bataille (Paris), F. Galacteros (Creteil), R. Girot (Paris), K. Bouzid (Rowba), D. Labie (Paris), A. Lambilliotte (Lille), S. Lemerle (Creteil), M. Malou (Annaba), M. Montalembert (Paris), M. Nathanson (Bondy), G. Noseda (Paris), C. Olivier (Colombes), E. Questiaux (Aulnay Sous Bois), B. Quinet (Paris), Ph. Reinert (Creteil), J. Rosa (Creteil), G. Tchernia (Kremlin Bicetre), D. Vanhaeke (Paris), B. Vignes (Montmorency), P. Zamet (Montmorency)