Current issues in blood transfusion for sickle cell disease

Although blood transfusion has been felt to be a beneficial therapy for sickle cell disease (SCD) since the 1950s, associated complications initially limited this therapy for these patients. With advances now reducing the side effects of transfusion and several landmark studies over the last decade clearly defining the efficacy for decreasing sickle cell morbidity, the indications for transfusion have increased. This review will discuss the indications, methods and goals of transfusion as well as complications and recent changes in transfusion therapy for SCD.

Recently studies have established the efficacy of transfusion for prevention of stroke, treatment of acute chest syndrome and perioperative transfusion management of SCD. Pulmonary hypertension is increasingly recognized as a significant source of morbidity and mortality and is an evolving indication for transfusion therapy. Phenotypically matching transfused blood has been shown to decrease alloimmunization, and genotyping for antigen matching may help match donors to patients in the future.

The increased use of transfusions may ultimately be balanced by hydroxyurea and other newer therapies developed as the complex pathophysiology of SCD is better understood; however, red cell transfusion is currently the most studied and accepted therapy for most acute and many chronic complications of SCD. Physicians caring for patients with sickle cell disease should be aware of the unique complications and transfusion requirements in this population.