Abstract
Hyperhemolysis syndrome (HHS) and delayed hemolytic transfusion reaction (DHTR) commonly occur in patients with sickle cell disease (SCD) and thalassemia, due to the need for recurrent red blood cell (RBC) transfusion, but rarely in patients with myelofibrosis. HHS is a life-threatening condition that occurs with or without DHTR, in which both transfused and autologous RBCs are destroyed. It needs a high clinical suspicion for diagnosis, especially when there is a drop in hemoglobin level to the level of pretransfusion of RBCs, accompanied by hyperbilirubinemia and reticulocytopenia. The management of HHS includes avoiding RBC transfusion, supportive care, and immunomodulatory therapy. We present a case of HHS with DHTR in a patient with primary myelofibrosis who was treated successfully with steroids and splenectomy.