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Research Article Free access | 10.1172/JCI110315

Deficiency of Suppressor T Cells in the Hyperimmunoglobulin E Syndrome

Raif S. Geha, Ellis Reinherz, Donald Leung, Kelly T. McKee Jr., Stuart Schlossman, and Fred S. Rosen

Divisions of Allergy and Immunology, Children's Hospital Medical Center, Sidney Farber Cancer Institute, Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts 02115

Department of Pediatrics, Vanderbilt University Hospital, Nashville, Tennessee 37232

Find articles by Geha, R. in: PubMed | Google Scholar

Divisions of Allergy and Immunology, Children's Hospital Medical Center, Sidney Farber Cancer Institute, Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts 02115

Department of Pediatrics, Vanderbilt University Hospital, Nashville, Tennessee 37232

Find articles by Reinherz, E. in: PubMed | Google Scholar

Divisions of Allergy and Immunology, Children's Hospital Medical Center, Sidney Farber Cancer Institute, Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts 02115

Department of Pediatrics, Vanderbilt University Hospital, Nashville, Tennessee 37232

Find articles by Leung, D. in: PubMed | Google Scholar

Divisions of Allergy and Immunology, Children's Hospital Medical Center, Sidney Farber Cancer Institute, Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts 02115

Department of Pediatrics, Vanderbilt University Hospital, Nashville, Tennessee 37232

Find articles by McKee, K. in: PubMed | Google Scholar

Divisions of Allergy and Immunology, Children's Hospital Medical Center, Sidney Farber Cancer Institute, Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts 02115

Department of Pediatrics, Vanderbilt University Hospital, Nashville, Tennessee 37232

Find articles by Schlossman, S. in: PubMed | Google Scholar

Divisions of Allergy and Immunology, Children's Hospital Medical Center, Sidney Farber Cancer Institute, Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts 02115

Department of Pediatrics, Vanderbilt University Hospital, Nashville, Tennessee 37232

Find articles by Rosen, F. in: PubMed | Google Scholar

Published September 1, 1981 - More info

Published in Volume 68, Issue 3 on September 1, 1981
J Clin Invest. 1981;68(3):783–791. https://doi.org/10.1172/JCI110315.
© 1981 The American Society for Clinical Investigation
Published September 1, 1981 - Version history
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Abstract

The status of suppressor T cells (Ts) was assessed in seven children with the hyper IgE syndrome (recurrent staphylococcal infections, eczematous skin rash, and elevated serum IgE) to determine whether a deficiency in Ts is associated with increased IgE synthesis. When circulating T cells and their subsets were enumerated with the aid of monoclonal antibodies that identify T cells (T3), helper/inducer T cells (T4), and suppressor/cytotoxic T cells (T8), there was a selective deficiency of T3+ cells (51.7±11.2% vs. 66±5% for normal controls) and of T8+ cells (7.5±4.4% vs. 22±4% for normal controls) but not of T4+ cells (36.5±7.5% vs. 37±3% for normal controls).

Suppressor T cell function was assessed by examining the ability of mononuclear cells incubated for 48 h with concanavalin A to suppress the proliferation of fresh autologous mononuclear cells in response to the mitogens phytohemagglutinin and pokeweed mitogen. All seven patients were severely deficient in concanavalin A-inducible suppressor cells.

In vitro de novo synthesis of IgE in 6-d cultures of peripheral blood lymphocytes was measured in four patients by a solid-phase radioimmunoassay. Mononuclear cells from all four patients synthesized spontaneously increased quantities of IgE in vitro (4,950±3,760 pg/106 cells vs. 250±215 pg/106 cells for eight normal controls). IgE synthesis was suppressed by the addition of parental T cells to the culture. Elimination of the T8+ subset, but not of the T4+ subset, by complement-dependent lysis resulted in the loss of the capacity of parental T cells to suppress IgE synthesis.

These results suggest that a deficiency of Ts underlies the elevated IgE levels observed in the hyper IgE syndrome.

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