Abnormalities of Immunoregulatory T Cells in Disorders of Immune Function

Ellis L. Reinherz, Arye Rubinstein, Raif S. Geha, Anthony J. Strelkauskas, Fred S. Rosen, Stuart F. Schlossman

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153 Scopus citations


We studied a five-year-old girl with several autoimmune disorders and a 16-year-old boy with acquired agammaglobulinemia to determine whether aberrations of immunoregulatory T cells could explain some instances of immunodeficiency or autoimmunity. The normal peripheral blood T-cell population, as defined by specific heteroantiserums, is 20 per cent TH2+ and 80 per cent TH2-. Human suppressor cells are TH2+, whereas helper cells are TH2-. In addition, each subset expresses la antigens upon activation. Our patient with autoimmune disease had no demonstrable TH2+ cells, and her lymphocytes could not be induced to suppress. Her circulating T cells were of an activated-helper phenotype, i.e., [mi],Ia+. In contrast, in the boy with agammaglobulinemia, the T-cell population was predominantly of an activated-suppressor phenotype, i.e., [mi],Ia+. This patient's T cells abrogated both his own and his histoidentical brother's B-cell secretion of immunoglobulins. We conclude that the characterization of T cells may provide insight into the causes of a number of abnormal immune states in man. (N Engl J Med 301:1018–1022, 1979) THE human T-cell population is composed of distinct subsets with unique functions.1 2 3 4 5 6 A delicate balance between effector and regulatory cells is required for immune homeostasis.7,8 Aberrations in immunoregulatory T-cell subsets have been shown to exist in a number of human diseases.7 8 9 For example, patients with active juvenile rheumatoid arthritis lack a regulatory T-cell subset, termed JRA+, at a time when their serum contains autoantibodies reactive with this subset of cells.7 In the absence of this immunoregulatory influence, marked hyperglobulinemia develops in these patients. Recently, we have shown that patients with acute graft-versus-host disease after bone-marrow transplantation lack the TH.

Original languageEnglish (US)
Pages (from-to)1018-1022
Number of pages5
JournalNew England Journal of Medicine
Issue number19
StatePublished - Nov 8 1979

ASJC Scopus subject areas

  • Medicine(all)


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