Thalassemia: A Review

Abstract

Thalassemia is a group of inherited diseases of the blood that affect a person's ability to produce hemoglobin, resulting in anemia. Around 100, 000 cases of thalassemia'sare found every year in the new born babies. Chances of occurrence of these cases are more among people of Italian, Greek, Middle Eastern, Southern Asian and African Ancestry. Thereare two main types of are called alpha and beta, depending on which part of an oxygen-carrying protein in the red blood cells is lacking. Both types of are inherited in the same manner. The disease is passed to children by parents who carry the mutated gene. A child who inherits one mutated gene is a carrier, which is sometimes called thalassemia trait. Most carriers lead completely normal, healthy lives. Diagnosis is usually done by hematologic tests, hemoglobin electrophoresis, and DNA analysis. Individuals with severe require blood transfusion, drug therapy i.e. deferoxamine, deferasirox, deferiprone, and bone marrow transplant. Bone Marrow Transplant (BMT) is still remains the only definitive cure available for patients with Thalassemia. The present paper discuss about the various types of Thalassemia's, their etiology, pathophysiology, clinical features, complication, prevention and treatment.