Pathologic findings in 76 autopsy cases of thalassemia.

Pathologic findings in six beta-thal major, 58 beta-thal Hb E, and 12 Hb H patients were presented. The causes of death were mainly infection in the first and second decades and cardiac failure in older patients. There was accelerated intramedullary and extramedullary erythropoiesis, with a few cases showing focal fibrosis, some with decreased cellularity and occasional atypia. Iron deposition mirrored that of thal patients in the European and American literature, apart from minimal cardiac siderosis, and the correlation between the amount of iron deposited and dysfunction of the particular organ was not always apparent. Patients with Hb H disease showed a less extensive iron deposition and organ pathology than those with the other two types of thalassemia.