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Hyperhemolytic transfusion reaction (HHTR) is a serious and potentially life-threatening complication of red blood cell (RBC) transfusion and has been well described in patients with sickle cell disease (SCD) and non-SCD patients. Awareness of this condition is important because subsequent transfusion may exacerbate hemolysis and may lead to a chronic protracted course or even death. If hemolysis is rapid and severe, subsequent transfusion may be necessary. Additional transfusion has been given together with intravenous immunoglobulin (IVIG) and steroids. We report a patient with SCD presented with severe HHTR whose serum contained multiple RBC alloantibodies. On day 2 of admission, the hemoglobin level dropped to 47 g/L. Intravenous immunoglobulin and steroid therapy was commenced. The patient responded and further transfusion was avoided. Review of the literature identified 5 HHTR cases in which transfusion was withheld and IVIG/steroids prescribed. In all of these cases, anemia was corrected and hemolysis resolved without blood transfusion. The reasons why transfusion was withheld and IVIG/steroids treatment prescribed were explored. There is no indication for IVIG in the routine treatment of hemolytic transfusion reactions, but IVIG should be considered as an option for treatment of serious, life-threatening HHTR both in SCD and non-SCD patients. [Copyright &y& Elsevier]