The Role of Blood Transfusion in the Management of Sickle Cell Disease in Africa

Abstract

Blood transfusions are sometimes required, most usually to treat severe anaemia, in patients with sickle cell disease (SCD). There is general lack of appreciation by clinicians, of the sub-optimal or frankly harmful effects, of inappropriate transfusion in SCD. This article discusses the relevant pathophysiology of sickle cell disease and the appropriate indications for blood transfusion. The adverse effects of blood transfusion in SCD are also discussed, particularly the greater susceptibility than usual to alloimmunization, posttransfusion anaemia, transfusion transmissible infection and vascular and iron overload, among others. Various clinical situations are described in which there is need, either merely to restore oxygen carrying capacity, or reduce the proportion of circulating sickle cells or suppress the production of sickle haemoglobin. Types of transfusion appropriate to each of these clinical situations are suggested, and general guidelines are recommended for haemotherapy of SCD.

Keywords: Blood Transfusion; Sickle cell Disease; Africa