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Successful Treatment of Refractory IgA‐Mediated Autoimmune Hemolytic Anemia With Bortezomib

Year: 2025

Type: article

Abstract: Introduction: IgA-mediated autoimmune hemolytic anemia (AIHA) is a rare condition associated with severe hemolysis and limited therapeutic response. Bortezomib, a proteasome inhibitor, targets plasma cells responsible for autoantibody production. Here, we describe a case of refractory IgA-mediated AIHA in a 13-year-old boy presenting with severe hemolysis, who was successfully treated with bortezomib. Methods: Blood samples were collected at different time points throughout the disease course for immunohematology testing. Results: The patient showed significant hematologic improvement following four doses of Bortezomib with reduction in hemolysis and recovery of hemoglobin levels. Laboratory tests revealed complement-negative, Coombs-positive blood tests combined with altered RBC morphology. Phagocytosis of patient's RBC was absent at all timepoints. Notably, despite hematologic improvement, IgA-positive RBC remained present, accompanied by compensated hemolysis. Conclusions: : The authors have confirmed clinical trial registration is not needed for this submission.

Source: eJHaem

DOI: 10.1002/jha2.70162