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.
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.
Authors: Ishan Patel; Mihir Odak; Steven Douedi; Abbas Alshami; Vandan D Upadhyaya; Mohammad Hossain; Madhurima Anne; Swapnil V Patel Journal: Eur J Case Rep Intern Med Date: 2021-08-07
Authors: Stella T Chou; Mouaz Alsawas; Ross M Fasano; Joshua J Field; Jeanne E Hendrickson; Jo Howard; Michelle Kameka; Janet L Kwiatkowski; France Pirenne; Patricia A Shi; Sean R Stowell; Swee Lay Thein; Connie M Westhoff; Trisha E Wong; Elie A Akl Journal: Blood Adv Date: 2020-01-28
Authors: Satheesh Chonat; Maa-Ohui Quarmyne; Caroline M Bennett; Christina L Dean; Clinton H Joiner; Ross M Fasano; Sean R Stowell Journal: Haematologica Date: 2018-05-24 Impact factor: 11.047