B-cell and T-regulatory cell dysfunction in six Chinese children with hypogammaglobulinaemia

Eur J Pediatr. 1993 May;152(5):409-13. doi: 10.1007/BF01955899.

Abstract

We report six Chinese boys with hypogammaglobulinaemia. All but one had very low or undetectable circulating B-lymphocytes, two had reversed CD4/CD8 ratios (in one of whom this latter became normal), one had reduced lymphocyte proliferative responses to concanavalin A and pokeweed mitogen and three had deficient responses to OKT3. Generation of antibody-secreting cells in response to pokeweed mitogen was markedly defective in all patients. Co-cultures of purified lymphocyte subsets from the patients with those of normal donors revealed that in addition to B-cell deficiency seen in all patients, two had T-helper cell deficiency and two had T-suppressor cell hyperactivity. One of the latter patients was treated with cimetidine in an attempt to ablate histamine type 2 receptor-bearing suppressor cells: the absolute number of such cells was temporarily reduced but there was no concurrent correction of the functional hyperactivity. These studies point to the variable nature of T-regulatory cell deficiencies in hypogammaglobulinaemia.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Agammaglobulinemia / drug therapy
  • Agammaglobulinemia / immunology*
  • B-Lymphocytes / drug effects
  • B-Lymphocytes / immunology*
  • CD4-CD8 Ratio
  • Child
  • Child, Preschool
  • China
  • Concanavalin A / pharmacology
  • Concanavalin A / therapeutic use
  • Humans
  • Infant
  • Leukocytes, Mononuclear / drug effects
  • Leukocytes, Mononuclear / immunology
  • Lymphocyte Activation
  • Male
  • Muromonab-CD3 / pharmacology
  • Muromonab-CD3 / therapeutic use
  • Pokeweed Mitogens / pharmacology
  • Pokeweed Mitogens / therapeutic use
  • T-Lymphocytes, Helper-Inducer / drug effects
  • T-Lymphocytes, Helper-Inducer / immunology*
  • T-Lymphocytes, Regulatory / drug effects
  • T-Lymphocytes, Regulatory / immunology*

Substances

  • Muromonab-CD3
  • Pokeweed Mitogens
  • Concanavalin A