Abnormalities of Immunoregulatory T Cells in Disorders of Immune Function

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Abstract
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)