Recommendations for the use of red blood cell exchange in sickle cell disease

Sickle cell disease (SCD) is a genetic disorder characterised by a single mutation of the beta globin gene, causing the production of an abnormal haemoglobin called sickle haemoglobin (HbS). In its deoxygenated form, HbS polymerises, causing major rheological disorders, which presents clinically as periodic vaso-occlusive crises, chronic haemolysis and chronic vascular dysfunction. Patients often resort to a background treatment, and transfusion remains the cornerstone in the management of the disease, significantly reducing morbidity and mortality. The aim of red blood cell exchange (RBCX) is to improve tissue oxygenation by increasing haemoglobin levels while lowering HbS levels. RBCX can be performed by manual or automated exchange, and each technique has its own set of advantages and disadvantages. This article will outline the transfusion indications for the main complications of SCD, as well as the most appropriate strategy to use.