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Life‐threatening delayed hyperhemolytic transfusion reaction in a patient with sickle cell disease: effective treatment with eculizumab followed by rituximab
Author(s) -
Boonyasampant Mark,
Weitz Ilene C.,
Kay Brian,
Boonchalermvichian Chaiyaporn,
Liebman Howard A.,
Shulman Ira A.
Publication year - 2015
Publication title -
transfusion
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.045
H-Index - 132
eISSN - 1537-2995
pISSN - 0041-1132
DOI - 10.1111/trf.13144
Subject(s) - eculizumab , medicine , rituximab , hemolysis , cold agglutinin disease , immunology , autoantibody , antibody , complement system , plasmapheresis , cold agglutinin , hemolytic anemia , gastroenterology
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°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.