BACKGROUND: Hemolytic transfusion reactions are a rare, yet serious complication of red blood cell (RBC) transfusion. Patients with sickle cell disease (SCD) are at an increased risk for such reactions, because they are prone to make alloantibodies against transfused RBCs, complicating this integral part of their disease management. These reactions may be missed, and the patient's state may be attributed to vaso-occlusive crisis (VOC), with misguided therapy ensuing.CASE REPORT: Herein, we report the clinical course of a patient with complex SCD with a delayed hemolytic transfusion reaction, followed by multiple acute hemolytic transfusion reactions mistaken for severe VOC. These reactions were diagnosed on retrospective review of the patient's clinical course in consult with the transfusion medicine service. Optimal immunosuppressive therapy ensued and resulted in the stabilization of the anemia and hemolysis laboratory values. Despite these efforts, the patient died 2 months after admission.
CONCLUSION:This case provides insight into some of the challenges of managing patients with SCD with multiple comorbidities. Hemolytic transfusion reactions can be difficult to diagnose and may be overlooked in patients with SCD with severe VOC. ABBREVIATIONS: AHTR = acute hemolytic transfusion reaction; ARC = absolute reticulocyte count; CAA = cold autoantibody; CHF = congestive heart failure; DAT = direct antiglobulin test; DHTR = delayed hemolytic transfusion reaction; DVT = deep venous thrombosis; Hb = hemoglobin; IAT = indirect antiglobulin test; IRL = immunohematology reference laboratory; LDH = lactate dehydrogenase; PEG = polyethylene glycol; RBC = red blood cell; SCD = sickle cell disease; TB = total bilirubin; VOC = vaso-occlusive crisis.