Transfusional iron overload and iron chelation therapy in thalassemia major and sickle cell disease

Abstract

KEY POINTS Thalassemia major is caused by defects in the synthesis of one or more of the globin subunits of hemoglobin, resulting in variable phenotypes. The yearly incidence of symptomatic individuals is estimated at 1 in 100,000 people throughout the world (22,989 new births) and 1 in 10,000 people in the European Union. Patients with thalassemia, being transfusion-dependent and having a hyperactive marrow, accumulate iron in tissues. The worldwide birth rate of individuals with symptomatic sickle cell disease (SCD) is approximately 2.2 per 1000 births. However, the disease incidence varies between ethnic groups. Blood transfusions may be required in both acute and chronic complications of SCD. SCD and thalassemia major differ in iron-loading patterns and in the prevalence of ironinduced organ damage