title: Gamma-delta T cells in Chediak-Higashi syndrome.
abstract: Lymphocytes from children with Chediak-Higashi syndrome (CHS) have impaired natural killer (NK) activity and lack antibody-dependent cell-mediated cytotoxicity. Study of T cells bearing an alternate T cell receptor comprised of gamma- and delta-chains, which typically demonstrate NK activity in vitro, was undertaken in CHS patients. We demonstrate that the cellular machinery for lysis of target cells in vitro is present in CHS-derived gamma delta T cell clones. We also show that the proportion of gamma delta T cells among peripheral blood mononuclear cells is significantly increased in CHS, the first example of a specific immunodeficiency disorder with a relative expansion of these T cells.
authors.affiliation: Department of Medicine, LSU Medical Center, Shreveport.
authors.forename: R F
authors.initials: RF
authors.lastname: Holcombe
authors.forename: R
authors.initials: R
authors.lastname: van de Griend
authors.forename: S L
authors.initials: SL
authors.lastname: Ang
authors.forename: R L
authors.initials: RL
authors.lastname: Bolhuis
authors.forename: J G
authors.initials: JG
authors.lastname: Seidman
mesh_terms: D002460:Cell Line; D002478:Cells, Cultured; D002609:Chediak-Higashi Syndrome / Q000097:blood / Q000276:immunology*; D002648:Child; D002999:Clone Cells; D003602:Cytotoxicity, Immunologic; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D012016:Reference Values; D013601:T-Lymphocytes / Q000276:immunology*