Abstract
Between 1983 and 1990 we treated 53 patients for congenital combined immunodeficency by bone marrow transplantation. 50 patients had SCID including 5 with ADA deficency and 3 with Omenn syndrome, 2 were treated for bare lymphocyte syndrome, two siblings suffered from a functional T-cell defect. The overall survival was 67% with a mean follow up of 4.2 years. After HLA - identical transplantation 7/9 (77.7%) and after haploidentical transplantation 29/45 (64.4%) survived. After haploidentical transplantation with T - cell depletion and without pretransplant conditioning, GvHD occured in 1/23, after pretreatment with ATG in 1/5 and after conditioning with busulfan and cyclophosphamide in 7/17 pts.. There was a slightly higher early mortality in patients with conditioning, but no difference in long time survival. However conditioned patients had a benefit in respect to B - cell reconstitution. The occurence of maternally derived lymphocytes (40% of pts.) had no influence on survival. The outcome of all these patients seems mainly influenced by their clinical state prior to BMT.
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Hartmann, W., Friedrich, W. 101 BONE MARROW TRANSPLANTATION IN CONGENITAL IMMUNODEFICENCY STATES – ULM EXPERIENCE. Pediatr Res 30, 645 (1991). https://doi.org/10.1203/00006450-199112000-00131
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DOI: https://doi.org/10.1203/00006450-199112000-00131