Consensus document for transfusion-related iron overload

Abstract

B LOOD TRANSFUSION is a common treatment option in patients with sickle cell disease (SCD). Stroke and other sicklingrelated complications that are characteristic of the disease occur less frequently with blood transfusions and hence provide the rationale for the use of transfusion in treatment of patients with SCD. An adverse consequence of repeated blood transfusions in patients with SCD is transfusion-related iron overload, which ultimately results in end-organ damage caused by accumulation of excessive tissue iron. Management of iron overload involves two fundamental approaches. The first approach is to minimize iron uptake by more judicious use of transfusions or to use alternate technologies such as exchange transfusions (manual or erythrocytapheresis) to reduce the rate of total body iron buildup. The second approach is to use iron chelation therapy to reduce total body iron stores. A team of practitioners and researchers skilled in identification and management of transfusion-related iron overload in patients with SCD (or the thalassemias) held a 2-day conference, the goal of which was to provide guidelines for management of blood transfusion and transfusion-related iron overload in patients with SCD. The process included a series of presentations, which are published in this supplement. The discussions included the use of blood transfusions in patients with SCD, blood product use, administration techniques, assessment of iron overload, alternative methods for assessing total body iron stores, management options for iron overload, and chelation therapy compliance. This document summarizes several areas of consensus for treatment guidelines; however, some issues remain controversial and are discussed in the individual presentations in this supplement. These issues still need further research before overall consensus can be reached.