Complications in Transfusion–dependent Patients of ß-thalassemia Major: a Review

ßthalassemia is an inherited disorder of hemoglobin synthesis characterized by deficient synthesis of the ß-globin chain that causes severe anemia. Over the years, the combination of hypertransfusion and chelation therapy has significantly increased the survival of patients of ß-thalassemia. At the same time, there has been an increase in the frequency of complications, mainly caused by iron overload. These include cardiac disorders which are the main determinants of survival. Endocrine complications include hypogonadism, diabetes mellitus, hypothyroidism, hypoparathyroidism and hypoadrenalism. Low bone mineral density and trace element deficiencies are among the metabolic complications seen in chronically transfused patients of ßthalassemia whereas hepatic problems and neuropsychologic disorders are also common.