title: Severe combined immunodeficiency with natural killer-cell predominance: abrogation of graft-versus-host disease and immunologic reconstitution with HLA-identical bone marrow cells.
abstract: A 3 1/2-month-old infant with severe combined immunodeficiency was found to have an unusual blood lymphocyte phenotype. Thirty percent of her cells formed rosettes with sheep erythrocytes, but only 7.9% reacted with the pan T monoclonal antibody OKT3, and 5% reacted with an antibody (OKT4)-recognizing T-helper cells. Surprisingly 19.4% of her cells reacted with an antibody (OKT8)-recognizing T-suppressor cells and 94% reacted with OKT10 . Few reacted with other monoclonal antibodies detecting cellular activation antigens. Despite absence of T or B cell function, her monocyte-depleted blood lymphocytes caused a high degree of specific lysis of 51Cr-labeled K562 erythromyeloid cells in a natural killer-cell assay. Most of her lymphocytes were large and had azurophilic granules and a monocytoid nucleus. Because she had received a nonirradiated, unrelated red-cell transfusion 3 days earlier, 4.8 X 10(9) nucleated bone-marrow cells from her HLA-identical brother were given shortly after admission. Two days later a graft-versus-host reaction began but subsided completely within 3 days. On day 15 posttransplantation, a profuse secretory diarrhea began, accompanied by a rise in her serum IgE from 4 to 3000 IU. With engraftment, the number of T10+ cells and natural killer-cell function fell to normal, and full immunologic reconstitution was achieved.
mesh_terms: D000911:Antibodies, Monoclonal / Q000276:immunology; D016026:Bone Marrow Transplantation; D002678:Chimera; D004802:Eosinophilia / Q000276:immunology; D005260:Female; D006086:Graft vs Host Disease / Q000628:therapy*; D006648:Histocompatibility; D006801:Humans; D007073:Immunoglobulin E / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000235:genetics / Q000276:immunology*; D007223:Infant; D007621:Karyotyping; D007694:Killer Cells, Natural / Q000276:immunology*; D008213:Lymphocyte Activation; D010641:Phenotype; D013601:T-Lymphocytes / Q000145:classification; D050378:T-Lymphocytes, Regulatory / Q000276:immunology
chemical_list: D000911:Antibodies, Monoclonal; D007073:Immunoglobulin E