A Review of Iron-Depleting Drugs in Patients with Thalassemia Dependent on Blood Transfusions

Thalassemia syndromes are the most common inherited hemoglobinopathies in the world characterized by various degrees of defective production of the alpha or beta globin chains. Impairment in alpha chain or beta chain synthesis leads to alpha thalassemia and beta thalassemia syndromes respectively.
Iran is one of the countries located on the thalassemia belt therefore this disease is significant in our country. The most important component of treatment in beta thalassemia is packed red blood cell transfusion. Thalassemic patients need lifelong transfusions and the consequence of these repeated transfusions is iron accumulation in different organs, such as heart, liver and endocrine gland. Iron overload is one of the major causes of morbidity in patients with thalassemia major necessitating iron chelation drugs to improve survival of patients. Three iron chelating agents are currently commercially available: deferoxamine (DFO), deferiprone (DFP), and deferasirox (DFX). DFO is the first chelating agent that became available. DFP is the first oral iron chelator and DFX is the most recently approved chelator for transfusional hemosiderosis. The purpose of this article is to summarize the main pharmacokinetic characteristics and side effects of iron chelator drugs in the field of thalassemia.