Life-threatening delayed hyperhemolytic transfusion reaction in a patient with sickle cell disease: effective treatment with eculizumab followed by rituximab

被引:40
作者
Boonyasampant, Mark [1 ]
Weitz, Ilene C. [2 ]
Kay, Brian [1 ]
Boonchalermvichian, Chaiyaporn [2 ]
Liebman, Howard A. [2 ]
Shulman, Ira A. [1 ]
机构
[1] Los Angeles Cty Univ Southern Calif, Med Ctr, Dept Pathol, Los Angeles, CA USA
[2] Univ So Calif, Keck Sch Med, Dept Med, Jane Anne Nohl Div Hematol, Los Angeles, CA 90033 USA
关键词
COMPLEMENT INHIBITOR ECULIZUMAB; IH;
D O I
10.1111/trf.13144
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
BACKGROUNDHyperhemolysis in sickle cell disease is a rare and potentially life-threatening complication of transfusion. STUDY DESIGN AND METHODSIn this article we report a case of delayed hemolytic transfusion reaction with resultant hyperhemolysis triggered by an anti-IH autoantibody with alloantibody behavior. RESULTSThe 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. CONCLUSIONSEculizumab, 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.
引用
收藏
页码:2398 / 2403
页数:6
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