title: Phenotype and function of engrafted maternal T cells in patients with severe combined immunodeficiency.
abstract: Engrafted maternal T cells from two patients with severe combined immunodeficiency (SCID) and graft-vs-host disease (GVHD) were characterized for surface phenotype, function, and ecto-5'-nucleotidase (ecto-5'-NT) activity. The majority of engrafted T cells from both patients were T6-, T3+, and Ia+; the ratio of T4+:T8+ cells varied from 0.89 to 3.1 for Patient 1 and was 0.17 for Patient 2. The sum of T4+ + T8+ cells was greater than the number of T3+ cells, and approximately one-third of the patients' T cells were T3-. Two-color immunofluorescent staining showed that one-third of the T cells from Patient 1 had a novel cell surface phenotype (T6-, T3-, T4+, T8+) that was not previously described. T cells from Patient 1 failed to proliferate in response to allogeneic cells or specific antigen and provided little help for PWM-driven Ig synthesis in vitro. However, they did suppress Ig synthesis in vitro and proliferate in response to PHA and Con A; thus they appeared to be more mature than the T cells of Patient 2 and of most previously reported patients with SCID and maternal T cell grafts. Both patients lacked detectable lymphocyte ecto-5'-NT activity, suggesting that either the ecto-5'-NT activity of maternal T cells is lost after engraftment or that a specific subset(s) of ecto-5'-NT-negative maternal T cells predominates in infants with SCID and GVHD. Thus, in vitro T cell function and the proportions of T cells bearing T4 and T8 may vary in SCID patients with maternal T cell grafts. However, the presence of the Ia antigen and the absence of ecto-5'-NT activity may be consistent features of activated maternal T cells responsible for GVHD.
mesh_terms: D015720:5'-Nucleotidase; D000328:Adult; D000954:Antigens, Surface / Q000032:analysis; D005260:Female; D006086:Graft vs Host Disease / Q000209:etiology / Q000276:immunology*; D006801:Humans; D007112:Immunity, Maternally-Acquired; D007136:Immunoglobulins / Q000096:biosynthesis; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000276:immunology*; D007223:Infant; D007231:Infant, Newborn; D008213:Lymphocyte Activation; D007959:Lymphocyte Culture Test, Mixed; D008297:Male; D009708:Nucleotidases / Q000378:metabolism; D010641:Phenotype; D011247:Pregnancy; D013601:T-Lymphocytes / Q000201:enzymology / Q000276:immunology*; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology; D014373:Tuberculin / Q000276:immunology
chemical_list: D000954:Antigens, Surface; D007136:Immunoglobulins; D014373:Tuberculin; D009708:Nucleotidases; D015720:5'-Nucleotidase