Boonyasampant Mark, Weitz Ilene C, Kay Brian, Boonchalermvichian Chaiyaporn, Liebman Howard A, Shulman Ira A
Department of Pathology, Los Angeles County-University of Southern California Medical Center.
Jane Anne Nohl Division of Hematology, Department of Medicine, Keck School of Medicine, Los Angeles, California.
Transfusion. 2015 Oct;55(10):2398-403. doi: 10.1111/trf.13144. Epub 2015 May 18.
Hyperhemolysis in sickle cell disease is a rare and potentially life-threatening complication of transfusion.
In this article we report a case of delayed hemolytic transfusion reaction with resultant hyperhemolysis triggered by an anti-IH autoantibody with alloantibody behavior.
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.
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.
镰状细胞病中的高溶血是一种罕见且可能危及生命的输血并发症。
在本文中，我们报告了一例由具有同种抗体行为的抗-IH自身抗体引发的迟发性溶血性输血反应并导致高溶血的病例。
抗-IH在室温以及37°C时均有反应，但与自身红细胞的反应较弱。初始冷凝集素效价为512。使用补体5（C5）抑制性抗体依库珠单抗后，严重的、危及生命的血管内溶血迅速且显著减轻。随后使用利妥昔单抗治疗抑制了自身/同种冷凝集素。
依库珠单抗是一种强效的C5抑制性抗体，当给予足够剂量以完全阻断补体C5的激活时，它可以快速有效地治疗高溶血性输血反应。
