Focal neurological deficits in children with beta-thalassemia major

The hematologic disorder ?-thalassemia major is relatively common in Southern Italy. Stroke is a well described, though infrequently reported, complication of this disorder. We now report our experience regarding 300 children with ?-thalassemia major examined at the University of Catania, Italy, over a 20-year period. We encountered 9 patients (3%; 3 males, 6 females) with ?-thalassemia major who had hemorrhagic stroke. Two groups of patients can be identified: group 1 (2 patients 22%) with early-onset post-transfusion hemorrhage and group 2 (7 patients 77%) with delayed post-transfusion hemorrhage. In the first group, the hemorrhage occurred within 48 hours 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, suggesting prolonged chronic anemia might play a role in the hemorrhage.