Endocrinopathies in patients with thalassemias

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

Principles of blood transfusion in sickle cell anemia

Sickle cell anemia [SCA] is one of the commonly inherited hemoglobinopathies in the Kingdom of Saudi Arabia. It is characterized by periods of remissions and exacerbations called crises as well as certain pathological phenomenon such as acute chest syndrome, priapism, hepatopathy, and cerebrovascular stroke. Blood transfusion [BT] as therapy and prophylaxis in SCA, although was advocated as early as the 1940's, there are still debates regarding its benefits and risks. This is a review of the value of BT in patients with SCA with emphasis on the risks and benefits as well as guidelines towards safe BT