Key Points• Autoimmune diseases do occur after CBT in approximately 5% of patients.• Of these, AIHA or ITP were observed the most often and were treated with prednisone, CSA, and RTX.To describe the incidence, risk factors, and treatment of autoimmune diseases (ADs) occurring after cord blood transplantation (CBT), we analyzed both CBT recipients reported to EUROCORD who had developed at least 1 new AD and those who had not. Fifty-two of 726 reported patients developed at least 1 AD within 212 days (range, 27-4267) after CBT. Cumulative incidence of ADs after CBT was 5.0% ؎ 1% at 1 year and 6.6% ؎ 1% at 5 years. Patients developing ADs were younger and had more nonmalignant diseases (P < .001). ADs target hematopoietic (autoimmune hemolytic anemia, n ‫؍‬ 20; Evans syndrome, n ‫؍‬ 9; autoimmune thrombocytopenia, n ‫؍‬ 11; and immune neutropenia, n ‫؍‬ 1) and other tissues (thyroiditis, n ‫؍‬ 3; psoriasis, n ‫؍‬ 2; Graves disease, n ‫؍‬ 1; membranous glomerulonephritis, n ‫؍‬ 2; rheumatoid arthritis, n ‫؍‬ 1; ulcerative colitis, n ‫؍‬ 1; and systemic lupus erythematosus, n ‫؍‬ 1). Four patients developed 2 ADs (3 cases of immune thrombocytopenia followed by autoimmune hemolytic anemia and 1 Evans syndrome with rheumatoid arthritis). By multivariate analysis, the main risk factor for developing an AD was nonmalignant disease as an indication for CBT (P ‫؍‬ .0001). Hematologic ADs were most often treated with steroids, rituximab, and cyclosporine. With a median follow-up of 26 months (range, 2-91), 6 of 52 patients died as a consequence of ADs. We conclude that CBT may be followed by potentially life-threatening, mainly hematologic ADs. (Blood. 2013;121(6):1059-1064)

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