Endocrinopathies in patients with thalassemias

ABSTRACT

Thalassemia major is a hereditary disorder of hemoglobin synthesis resulting in severe anemia. Treatment consists of multiple blood transfusions, a complication of which is iron overload. Excessive iron is then deposited in almost all tissues but primarily in the liver, heart and the endocrine glands. Lately, desferrioxamine has been used as a chelating agent in an attempt to prevent the complications of tissue damage by iron deposition. Early introduction of the chelating agent to combat iron overload in vulnerable organs leads to improved life expectancy. However, these patients often present with multiple endocrine dysfunction such as growth failure, hypogonadism, abnormalities in glucose metabolism, hypothyroidism, hypoparathyroidism and less frequently hypoadrenalism. We briefly review the current status of endocrine gland abnormalities in patients with thalassemia major