Transfusion in Sickle cell disease: best practice and understanding of its utility

Abstract

Transfusion therapy remains an important treatment modality for patients with sickle cell disease (SCD). Transfusions are given not only to treat symptomatic anaemia, but also to decrease SCD‐related complications by lowering the percentage of circulating sickle red cells, thus decreasing blood viscosity and vaso‐occlusion. The strongest evidence of the effectiveness of transfusion is in primary and secondary stroke prophylaxis, where transfusion reduces the risk of overt stroke by about 90–70%, and in pre‐operative transfusion, where transfusion reduces the risk of acute chest syndrome by at least 30%. Most other indications for transfusion in SCD are based on expert opinion or less well‐controlled studies. In this brief overview, we discuss methods of transfusion, the evidence and expert opinion regarding various indications in sickle cell disease and our current pathophysiologic understanding of how transfusions may work in SCD.