title: Immunodeficiency associated with selective loss of helper/inducer T cells and hypogammaglobulinaemia in a child with intestinal lymphangiectasia.
abstract: A patient with intestinal lymphangiectasia (IL) was studied for his immunological abnormalities. The patient had hypoproteinaemia with severe hypogammaglobulinaemia. The results of lymphocyte subpopulation studies revealed a decrease of CD4+ cells and a decrease of surface immunoglobulin (sIg)-positive B lymphocytes. T cell functions determined by the proliferative responses against Concanavalin A (Con A) or phytohaemagglutinin (PHA) and by delayed type cutaneous hypersensitivity (DTH) response to purified protein derivatives (PPD) and PHA were normal. No immunoglobulin (Ig)-secreting cells were induced when his peripheral blood lymphocytes (PBL) were cultured with pokeweed mitogen (PWM). His T cells showed suppressor function to the PWM-induced differentiation of normal B cells. This suppressor activity was sensitive to 3000 rads irradiation. His B cells also failed to differentiate into Ig-producing cells with the help of normal T cells and PWM. Thus, in this patient, the decrease of Ig-synthesis in vitro could be attributed to suppressor T cells, lack of T helper cells and an intrinsic B cell defect. Therefore, this patient appears to have immunological abnormalities which differ from previously reported IL patients.
authors.affiliation: Department of Pediatrics, Kobe City General Hospital, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Yamamoto
authors.forename: T
authors.initials: T
authors.lastname: Tsutsui
authors.forename: M
authors.initials: M
authors.lastname: Mayumi
authors.forename: S
authors.initials: S
authors.lastname: Kasakura
mesh_terms: D000361:Agammaglobulinemia / Q000209:etiology; D002648:Child; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis / Q000096:biosynthesis; D007153:Immunologic Deficiency Syndromes / Q000209:etiology*; D008201:Lymphangiectasis, Intestinal / Q000150:complications* / Q000276:immunology; D008214:Lymphocytes / Q000145:classification; D008297:Male; D011504:Protein-Losing Enteropathies / Q000150:complications*; D013601:T-Lymphocytes / Q000276:immunology; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology*
chemical_list: D007136:Immunoglobulins