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

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

International Standard Serial Number (ISSN)

  • 0271-9142

Language

  • eng

Conference Location

  • Netherlands