Guidelines on Red Cell Transfusion in Sickle Cell Disease. Part I: Principles and Laboratory Aspects

Blood transfusion in the management of sickle cell disease (SCD) can be lifesaving and reduces disability. However, it may cause morbidity, including alloimmunisation and iron overload (Ballas 2001, Darbari, et al 2006, Rosse, et al 1990, Vichinsky, et al 1990), and mortality (Royal and Seeler 1978, Serjeant 2003). A paucity of randomised controlled clinical trials has resulted in wide variations in clinical practice. However, recent randomised studies have addressed some of the outstanding issues around indications to prevent some chronic complications (DeBaun, et al 2014) and to prevent perioperative acute complications, such as acute chest syndrome (Howard, et al 2013). We have reviewed the evidence and developed two linked guidelines on transfusion in SCD; Part I relates to general principles and laboratory aspects, whereas Part II addresses indications for transfusion in sickle cell disease. Here the term sickle cell disease refers to all genotypes of the disease and sickle cell anaemia to the homozygous state (SS). Abstract source