@article {4410,
	title = {Life-threatening delayed hyperhemolytic transfusion reaction in a patient with sickle cell disease: effective treatment with eculizumab followed by rituximab.},
	journal = {Transfusion},
	year = {2015},
	month = {2015},
	publisher = {(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,},
	address = {(C) 2015 John Wiley \& Sons, Ltd},
	abstract = {BACKGROUND: 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, Ltd},
	isbn = {0041-1132},
	author = {Boonyasampant, Mark and Weitz, Ilene and Kay, Brian and Boonchalermvichian, Chaiyaporn and Liebman, Howard and Shulman, Ira}
}
