title: Donor type natural killer cells after haploidentical T cell-depleted bone marrow stem cell transplantation in a patient with adenosine deaminase-deficient severe combined immunodeficiency.
abstract: T cell-depleted haploidentical (parental) bone marrow stem cell transplants are given to most infants with the syndrome of severe combined immunodeficiency (SCID) because they have no available HLA-identical sibling potential donors. Since they usually do not undergo cytoreduction prior to transplantation, these children later demonstrate mixed hematopoietic chimerism. Most often, T cells (but usually not B lymphocytes, macrophages, or other hematopoietic cells) can be shown to be of donor type. The origin of natural killer (NK) cells in such chimeras has not been reported. Two lymphocyte lines derived from the CD16+ fraction of an adenosine deaminase (ADA)-deficient male SCID's blood mononuclear cells (MNC) 13 months following maternal marrow stem cell transplantation demonstrated typical phenotypic and functional characteristics of NK cells after expansion. Karyotyping showed both lines to be XX. Thus, NK cell engraftment can occur in SCID infants who have not been conditioned, even when significant NK cell function is present before transplantation.
authors.affiliation: Department of Pediatrics, Duke University School of Medicine, Durham, North Carolina 27710.
authors.forename: A D
authors.initials: AD
authors.lastname: Gaines
authors.forename: S E
authors.initials: SE
authors.lastname: Schiff
authors.forename: R H
authors.initials: RH
authors.lastname: Buckley
mesh_terms: D000243:Adenosine Deaminase / Q000172:deficiency*; D000943:Antigens, Differentiation / Q000032:analysis; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis; D016026:Bone Marrow Transplantation / Q000276:immunology*; D017252:CD3 Complex; D018959:CD57 Antigens; D002678:Chimera / Q000276:immunology; D006801:Humans; D007108:Immune Tolerance / Q000276:immunology*; D007075:Immunoglobulin M / Q000032:analysis; D007165:Immunosuppression Therapy; D007223:Infant; D007694:Killer Cells, Natural / Q000276:immunology*; D008212:Lymphocyte Depletion; D008297:Male; D011948:Receptors, Antigen, T-Cell / Q000032:analysis; D011961:Receptors, Fc / Q000032:analysis; D017452:Receptors, IgG
chemical_list: D000943:Antigens, Differentiation; D000945:Antigens, Differentiation, T-Lymphocyte; D017252:CD3 Complex; D018959:CD57 Antigens; D007075:Immunoglobulin M; D011948:Receptors, Antigen, T-Cell; D011961:Receptors, Fc; D017452:Receptors, IgG; D000243:Adenosine Deaminase