Heterogeneity of immunological abnormalities in ataxia-telangiectasia

J Clin Immunol. 1983 Apr;3(2):135-41. doi: 10.1007/BF00915484.


Peripheral blood mononuclear cells from five patients with ataxia-telangiectasia were evaluated for their reactivity with a panel of monoclonal antibodies directed against T-cell subsets and for their in vitro functions in a pokeweed mitogen-induced immunoglobulin biosynthesis assay. All the patients had significantly reduced proportions of cells identified by monoclonal antibodies to subpopulations of T lymphocytes with helper activity (OKT4 and 5/9) and produced low amounts or no IgA and IgG in vitro. Immunoglobulin biosynthesis was increased by the addition of normal x-irradiated peripheral blood mononuclear cells in one of three patients, suggesting a helper T-cell deficiency in this patient and intrinsic B-cell defects in the other two. Two patients had increased proportions of cells identified by a monoclonal antibody to a subpopulation of T lymphocytes which includes suppressor T cells (OKT8), and their cells were able to suppress immunoglobulin biosynthesis by peripheral blood mononuclear cells from normal donors. These findings indicate heterogeneous disturbances of immunoregulatory mechanisms in ataxia-telangiectasia.

MeSH terms

  • Adolescent
  • Adult
  • Antibodies, Heterophile / physiology*
  • Antibodies, Monoclonal / physiology
  • Ataxia Telangiectasia / immunology*
  • B-Lymphocytes / physiology
  • Child
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Humans
  • Immunoglobulins / biosynthesis
  • In Vitro Techniques
  • Male
  • T-Lymphocytes, Helper-Inducer / physiology
  • T-Lymphocytes, Regulatory / physiology


  • Antibodies, Heterophile
  • Antibodies, Monoclonal
  • Immunoglobulins