Immunodeficiency associated with selective loss of helper/inducer T cells and hypogammaglobulinaemia in a child with intestinal lymphangiectasia

Clin Exp Immunol. 1989 Feb;75(2):196-200.

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.

Publication types

  • Case Reports

MeSH terms

  • Agammaglobulinemia / etiology
  • Child
  • Humans
  • Immunoglobulins / analysis
  • Immunoglobulins / biosynthesis
  • Immunologic Deficiency Syndromes / etiology*
  • Lymphangiectasis, Intestinal / complications*
  • Lymphangiectasis, Intestinal / immunology
  • Lymphocytes / classification
  • Male
  • Protein-Losing Enteropathies / complications*
  • T-Lymphocytes / immunology
  • T-Lymphocytes, Helper-Inducer / immunology*

Substances

  • Immunoglobulins