Erythrocyte autoantibodies in paediatric patients with sickle cell diseasereceiving transfusion therapy: frequency, characteristics and significance

The formation of erythrocyte autoantibodies following transfusion therapy h as been described in case reports and small series. To determine the freque ncy, serological characteristics, and clinical significance of this phenome non in paediatric patients with sickle cell disease, we analysed the patien t database at the Duke University Pediatric Hematology Clinic. We identifie d children who received multiple erythrocyte transfusions, then reviewed cl inical records to identify children who developed erythrocyte autoantibodie s in association with transfusions. Among 184 paediatric patients who recei ved multiple erythrocyte transfusions, 14 children (7.6%) developed warm (I gG) erythrocyte autoantibodies. Median transfusion exposure at the time of autoantibody formation was 24 erythrocyte units, range 3-341 units. The aut oantibody reacted as a panagglutinin in 11 cases but had anti-e specificity in three patients, Surface complement also was detected in five patients. Clinically significant haemolysis was documented in four patients, each of whom had both surface IgG and C3 detected. The development of erythrocyte a utoantibodies was associated with the presence of erythrocyte alloantibodie s. Formation of warm erythrocyte autoantibodies in association with transfu sions is not rare in paediatric patients with sickle cell disease. Clinicia ns should be aware of this complication and recognize that the presence of surface C3 is often associated with significant haemolysis.