Hyperhemolysis syndrome in a patient without a hemoglobinopathy, unresponsive to treatment with eculizumab

• DOI: 10.1111/trf.12876
• Publication Date: 2014-09-26T08:20:14Z
• Authors: Shruti Gupta; Andrew Fenves; Sandra Taddie Nance; David B. Sykes; Walter “Sunny” Dzik
• Keywords: Eculizumab; Hemoglobinopathy; Coombs test

Background Hyperhemolysis is a serious transfusion reaction, most often described in patients with hemoglobinopathies. characterized by the destruction of host red blood cells ( RBCs ), addition to donor , via an unknown mechanism. Study Design and Methods We present case 58‐year‐old woman treated human immunodeficiency virus normal hemoglobin Hb ) electrophoresis who developed hyperhemolysis setting delayed hemolytic reaction DHTR ). Results The patient was ABO group B had previously identified anti‐ F y b alloantibody. After – she found have E C w anti‐s, additional antibody unrecognized high‐frequency RBC alloantigen. Subsequent ‐compatible that were negative for s antigens resulted immediate intravascular hemolysis. In absence bleeding, her hematocrit Hct decreased 10.2%. An extensive serologic evaluation failed identify specificity antibody. Severe reactions also occurred despite pretransfusion conditioning eculizumab. clinical symptoms slowly improved after cessation transfusions treatment erythropoietin steroids. This demonstrates several noteworthy features including without disorder, development antigen, failure eculizumab prevent hemolysis transfusion. Conclusion not restricted Whether offers any benefit syndrome or prevention due alloantibodies remains uncertain.