title: Life-threatening delayed hyperhemolytic transfusion reaction in a patient with sickle cell disease: effective treatment with eculizumab followed by rituximab.
abstract: BACKGROUND
  Hyperhemolysis in sickle cell disease is a rare and potentially life-threatening complication of transfusion.
  
  
  METHODS
  In this article we report a case of delayed hemolytic transfusion reaction with resultant hyperhemolysis triggered by an anti-IH autoantibody with alloantibody behavior.
  
  
  RESULTS
  The anti-IH was reactive at room temperature as well as 37 °C, but only weakly reactive with autologous red blood cells. Initial cold agglutinin titer was 512. The profound, life-threatening, intravascular hemolysis was rapidly and dramatically reduced with the Complement 5 (C5) inhibitory antibody, eculizumab. The auto/allo cold agglutinin was subsequently suppressed with rituximab treatment.
  
  
  CONCLUSIONS
  Eculizumab, a potent C5 inhibitory antibody, can be a rapid and effective therapy for hyperhemolytic transfusion reactions when given in a sufficient dose to fully block the activation of complement C5.
authors.affiliation: Department of Pathology, Los Angeles County-University of Southern California Medical Center.
authors.forename: Mark
authors.initials: M
authors.lastname: Boonyasampant
authors.affiliation: Jane Anne Nohl Division of Hematology, Department of Medicine, Keck School of Medicine, Los Angeles, California.
authors.forename: Ilene C
authors.initials: IC
authors.lastname: Weitz
authors.affiliation: Department of Pathology, Los Angeles County-University of Southern California Medical Center.
authors.forename: Brian
authors.initials: B
authors.lastname: Kay
authors.affiliation: Jane Anne Nohl Division of Hematology, Department of Medicine, Keck School of Medicine, Los Angeles, California.
authors.forename: Chaiyaporn
authors.initials: C
authors.lastname: Boonchalermvichian
authors.affiliation: Jane Anne Nohl Division of Hematology, Department of Medicine, Keck School of Medicine, Los Angeles, California.
authors.forename: Howard A
authors.initials: HA
authors.lastname: Liebman
authors.affiliation: Department of Pathology, Los Angeles County-University of Southern California Medical Center.
authors.forename: Ira A
authors.initials: IA
authors.lastname: Shulman
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000628:therapy*; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D001787:Blood Group Incompatibility / Q000188:drug therapy*; D005260:Female; D006461:Hemolysis / Q000187:drug effects*; D006801:Humans; D007518:Isoantibodies / Q000097:blood; D000069283:Rituximab / Q000008:administration & dosage*; D065227:Transfusion Reaction
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D007518:Isoantibodies; D000069283:Rituximab; C481642:eculizumab