Normalization of the peripheral blood T cell receptor V beta repertoire after cultured postnatal human thymic transplantation in DiGeorge syndrome.

Published

Journal Article

Complete DiGeorge syndrome is an immunodeficiency disease characterized by thymic aplasia and the absence of functioning peripheral T cells. A patient with this syndrome was transplanted with cultured postnatal human thymic tissue. Within 5 weeks of transplantation, flow cytometry, T cell receptor V beta sequence analysis, and cell function studies showed the presence of oligoclonal populations of nonfunctional clonally expanded peripheral T cells that were derived from pretransplantation T cells present in the skin. However, at 3 months posttransplantation, a biopsy of the transplanted thymus showed normal intrathymic T cell maturation of host T cells with normal TCR V beta expression on thymocytes. By 9 months postransplantation, peripheral T cell function was restored and the TCR V beta repertoire became polyclonal, coincident with the appearance of normal T cell function. These data suggest that the transplanted thymus was responsible for the establishment of a new T cell repertoire via thymopoiesis in the chimeric thymic graft.

Full Text

Duke Authors

Cited Authors

  • Davis, CM; McLaughlin, TM; Watson, TJ; Buckley, RH; Schiff, SE; Hale, LP; Haynes, BF; Markert, ML

Published Date

  • March 1997

Published In

Volume / Issue

  • 17 / 2

Start / End Page

  • 167 - 175

PubMed ID

  • 9083893

Pubmed Central ID

  • 9083893

International Standard Serial Number (ISSN)

  • 0271-9142

Language

  • eng

Conference Location

  • Netherlands