Interaction of Transfusion and Iron Chelation in Thalassemias

Abstract

The relationship between blood transfusion intensity, chelatable iron pools, and extrahepatic iron distribution is described in thalassemia. Risk factors for cardiosiderosis are discussed with particular reference to the balance of transfusional iron loading rate and transferrin-iron utilization rate as marked by plasma levels of soluble transferrin receptors. Low transfusion regimens increase residual erythropoiesis allowing for apotransferrin-dependent clearance of non-transferrin-bound iron species otherwise destined for myocardium. The impact of transfusion rates on chelation dosing required for iron balance is also shown.

Keywords: Cardiosiderosis; Chelators; Erythropoiesis; Hepcidin; Iron; Iron overload; Soluble transferrin receptor; Transfusion.