EJHaem. 2025 Oct 25;6(6):e70162. doi: 10.1002/jha2.70162. eCollection 2025 Dec.
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 present case demonstrates the potential of bortezomib as a treatment option for refractory AIHA cases, particularly in children. Trial Registration: The authors have confirmed clinical trial registration is not needed for this submission.
PMID:41143283 | PMC:PMC12552891 | DOI:10.1002/jha2.70162
