Focal neurological deficits in children with beta-thalassemia major

The hematologic disorder beta-thalassemia major is relatively common in Sou thern Italy. Stroke is a well described, though infrequently reported. comp lication of this disorder. We now report our experience regarding 300 child ren with beta-thalassemia major examined at the University of Catania, Ital y, over a 20-year period. We encountered 9 patients (3 %; 3 males, 6 female s) with beta-thalassemia major who had hemorrhagic stroke. Two groups of pa tients can be identified: group 1 (2 patients 22 %) with early-onset post-t ransfusion hemorrhage and group Z (7 patients 77%) with delayed post-transf usion hemorrhage. In the first group, the hemorrhage occurred within 48 hou rs following blood transfusion. In the second group, hemorrhage occurred 7- 15 days from last transfusion. In 5 patients out of 7 of this second group the first transfusion and ictal event both occurred after age five, suggest ing prolonged chronic anemia might play a role in the hemorrhage.