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

Abstract

Iron overload is an inevitable consequence of blood transfusions and is often accompanied by increased iron absorption from the gut. Chelation therapy is necessary to prevent the consequences of hemosiderosis. Three chelators, deferoxamine, deferiprone, and deferasirox, are presently available and a fourth is undergoing clinical trials. The efficacy of all 3 available chelators has been demonstrated. Also, many studies have shown the efficacy of the combination of deferoxamine plus deferiprone as an intensive treatment of severe iron overload. Alternating chelators can reduce adverse effects and improve compliance. Adherence to therapy is crucial for good results.

Keywords: Deferasirox; Deferiprone; Deferoxamine; Iron chelation; Iron overload; Sickle cell disease; Thalassemia major.