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Thymic output, T-cell diversity, and T-cell function in long-term human SCID chimeras
Sarzotti-Kelsoe, M., Win, C. M., Parrott, R. E., Cooney, M., Moser, B. K., Roberts, J. L., Sempowski, G. D., & Buckley, R. H. (2009). Thymic output, T-cell diversity, and T-cell function in long-term human SCID chimeras. Blood, 114(7), 1445-1453. https://doi.org/10.1182/blood-2009-01-199323
Severe combined immunodeficiency (SCID) is a syndrome of diverse genetic cause characterized by profound deficiencies of T, B, and sometimes NK-cell function. Nonablative human leukocyte antigen-identical or rigorously T cell-depleted haploidentical parental bone marrow transplantation (BMT) results in thymus-dependent genetically donor T-cell development in the recipients, leading to long-term survival. We reported previously that normal T-cell numbers, function, and repertoire developed by 3 to 4 months after transplantation in SCID patients, and the repertoire remained highly diverse for the first 10 years after BMT. The T-cell receptor diversity positively correlated with T-cell receptor excision circle levels, a reflection of thymic output. However, the fate of thymic function in SCID patients beyond 10 to 12 years after BMT remained to be determined. In this greater than 25-year follow-up study of 128 patients with 11 different molecular types of SCID after nonconditioned BMT, we provide evidence that T-cell function, thymic output, and T-cell clonal diversity are maintained long-term.
