Autoimmune hemolytic anemia in patients with SCID after T cell-depleted BMand PBSC transplantation

We report a high incidence (19.5%) of autoimmune hemolytic anemia (AIHA) in 41 patients with SCID who underwent a T cell-depleted haploidentical trans plant, Other than infections, AIHA was the most common posttransplant compl ication in this patient cohort. Clinical characteristics and treatment of e ight patients who developed AIHA at a median of 8 months after the first T cell-depleted transplant are presented. All patients had warm-reacting auto antibodies, and two of eight had concurrent cold and warm autoantibodies. C linical course was most severe in two patients who had cold and warm autoan tibodies. Five patients received specific therapy for AIHA. Successful tape r off immunosuppressive therapy for AIHA coincided with T cell reconstituti on. Delayed reconstitution of T cell immunity, due to T cell depletion, imm unosuppressive conditioning and CsA, as well as paucity of regulatory T cel ls, are the likely explanations for the occurrence of AIHA in our patient c ohort. Screening of the population at risk may prevent morbidity and mortal ity from AIHA.