Kyoko Yoshihara, Saki Takahashi, Junko Ikemoto, Nobuto Utsunomiya, Mami Samori, Shinya Ohtsuka, Rie Murata, Satoshi Yoshihara
Autoimmune hemolytic anemia (AIHA) is a potentially life-threatening complication of lymphoid malignancies, and transfusion management can be particularly challenging in refractory cases. We report a case of angioimmunoblastic T-cell lymphoma complicated by severe, transfusion-refractory warm AIHA, in which red blood cell transfusion was paradoxically associated with a hyperhemolysis-like process and overt disseminated intravascular coagulation. Despite corticosteroid therapy and rituximab, repeated transfusions failed to produce sustained hemoglobin increments and were followed by worsening hemolysis and coagulopathy. Detailed immunohematologic evaluation demonstrated panreactive IgG autoantibodies with persistently positive direct antiglobulin testing and elevated polyethylene glycol-enhanced indirect antiglobulin test reactivity. Therapeutic plasma exchange was initiated as a rescue intervention, resulting in a progressive reduction in circulating autoantibody reactivity, restoration of transfusion efficiency, and stabilization of hemoglobin levels, despite persistent direct antiglobulin test positivity. Following effective lymphoma-directed chemotherapy, hemolysis resolved completely, and transfusion independence was achieved. This case highlights post-transfusion deterioration resembling hyperhemolysis in lymphoma-associated AIHA and underscores the potential role of therapeutic plasma exchange as a rescue and bridging therapy in life-threatening, transfusion-refractory disease.