Thymic output, T-cell diversity, and T-cell function in long-term human SCID chimeras.

Published

Journal Article

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.

Full Text

Duke Authors

Cited Authors

  • Sarzotti-Kelsoe, M; Win, CM; Parrott, RE; Cooney, M; Moser, BK; Roberts, JL; Sempowski, GD; Buckley, RH

Published Date

  • August 13, 2009

Published In

Volume / Issue

  • 114 / 7

Start / End Page

  • 1445 - 1453

PubMed ID

  • 19433858

Pubmed Central ID

  • 19433858

Electronic International Standard Serial Number (EISSN)

  • 1528-0020

Digital Object Identifier (DOI)

  • 10.1182/blood-2009-01-199323

Language

  • eng

Conference Location

  • United States