POSTTRANSFUSION HYPERHAEMOLYSIS IN A PATIENT WITH SICKLE-CELL DISEASE- USE OF STEROIDS AND INTRAVENOUS IMMUNOGLOBULIN TO PREVENT FURTHER RED-CELL DESTRUCTION

Delayed haemolytic transfusion reactions (DHTRs) are seen more frequen tly in patients with sickle cell disease (SCD) than in other groups of patients, and are characterised by a positive direct antiglobulin tes t and the appearance of previously undetected red blood cell (RBC) all oantibodies in the patient's serum. Recently a syndrome of post-transf usion hyperhaemolysis has been described in children with SCD, charact erised by destruction of both autologous and transfused RBCs with nega tive serological findings: continuation of RBC transfusion exacerbated haemolysis further. We describe a case of life-threatening posttransf usion hyperhaemolysis in an adult patient with SCD in whom severe anae mia necessitated further RBC transfusion, which was successfully perfo rmed in conjunction with intravenous immunoglobulin. This approach may be useful in the management of post-transfusion hyperhaemolysis in SC D as well as in the management of severe DHTRs.