Related Experiment Videos
Focal neurological deficits in children with beta-thalassemia major
G Incorpora1, F Di Gregorio, M A Romeo
1Division of Pediatric Neurology, University of Catania, Italy.
Insights
Hemorrhagic stroke is a rare complication in children with beta-thalassemia major. This study found that delayed post-transfusion hemorrhage occurred in 7 of 9 pediatric patients, suggesting chronic anemia may contribute.
Area of Science:
- Hematology
- Pediatric Neurology
Background:
- Beta-thalassemia major is a common hematologic disorder in Southern Italy.
- Stroke is an infrequent but recognized complication of beta-thalassemia major.
Observation:
- A 20-year study at the University of Catania examined 300 children with beta-thalassemia major.
- Nine patients (3%) experienced hemorrhagic stroke.
- Patients were categorized into early-onset (within 48 hours) and delayed (7-15 days) post-transfusion hemorrhage groups.
Findings:
- Delayed post-transfusion hemorrhage was more common, affecting 7 out of 9 patients.
- In 5 of these 7 patients, both the first transfusion and stroke occurred after age five.
- This suggests prolonged chronic anemia may be a risk factor for stroke in beta-thalassemia major.
Implications:
- Understanding hemorrhage timing is crucial for managing beta-thalassemia major patients.
- Further research into the role of chronic anemia in stroke pathogenesis is warranted.
- This highlights the importance of neurological monitoring in pediatric beta-thalassemia patients.
Abstract:
The hematologic disorder beta-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 beta-thalassemia major examined at the University of Catania, Italy, over a 20-year period. We encountered 9 patients (3%; 3 males, 6 females) with beta-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.