01913nas a2200193   4500008004100000020001400041024001700055245016300072210006900235260025500304520098700559100002401546700001701570700001501587700003501602700002001637700001701657856004501674       2015                        eng d  a0041-1132  awdn, 041736000aLife-threatening delayed hyperhemolytic transfusion reaction in a patient with sickle cell disease: effective treatment with eculizumab followed by rituximab.0 aLifethreatening delayed hyperhemolytic transfusion reaction in a  a(C) 2015 John Wiley & Sons, Ltdb(1)Department of Pathology, Los Angeles County-University of Southern California Medical Center,(2)Jane Anne Nohl Division of Hematology, Department of Medicine, Keck School of Medicine, Los Angeles, California,c20153 aBACKGROUND: Hyperhemolysis in sickle cell disease is a rare and potentially life-threatening complication of transfusion., STUDY DESIGN AND 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[degrees]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., (C) 2015 John Wiley & Sons, Ltd1 aBoonyasampant, Mark1 aWeitz, Ilene1 aKay, Brian1 aBoonchalermvichian, Chaiyaporn1 aLiebman, Howard1 aShulman, Ira  uhttps://notifylibrary.org/reference/4410