title: Normalization of the peripheral blood T cell receptor V beta repertoire after cultured postnatal human thymic transplantation in DiGeorge syndrome.
abstract: 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.
authors.affiliation: Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.
authors.forename: C M
authors.initials: CM
authors.lastname: Davis
authors.forename: T M
authors.initials: TM
authors.lastname: McLaughlin
authors.forename: T J
authors.initials: TJ
authors.lastname: Watson
authors.forename: R H
authors.initials: RH
authors.lastname: Buckley
authors.forename: S E
authors.initials: SE
authors.lastname: Schiff
authors.forename: L P
authors.initials: LP
authors.lastname: Hale
authors.forename: B F
authors.initials: BF
authors.lastname: Haynes
authors.forename: M L
authors.initials: ML
authors.lastname: Markert
mesh_terms: D001483:Base Sequence; D004062:DiGeorge Syndrome / Q000276:immunology / Q000378:metabolism / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008969:Molecular Sequence Data; D016377:Organ Transplantation; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000235:genetics / Q000276:immunology / Q000378:metabolism*; D016415:Sequence Alignment; D013950:Thymus Gland / Q000166:cytology / Q000637:transplantation*
chemical_list: D016693:Receptors, Antigen, T-Cell, alpha-beta