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

TL;DR

A 13-year-old boy with severe, treatment-resistant IgA-mediated autoimmune hemolytic anemia showed significant improvement after receiving bortezomib.

Contribution

This case demonstrates bortezomib's potential as a treatment for refractory IgA-mediated AIHA in children.

Findings

The patient showed significant hematologic improvement after four doses of bortezomib.

IgA-positive red blood cells remained present despite improvement, indicating ongoing disease activity.

Phagocytosis of red blood cells was absent at all timepoints.

Abstract

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.

Blood samples were collected at different time points throughout the disease course for immunohematology testing.

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.

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

Figures