Expansion of CD3+CD4-CD8- T cell population expressing high levels of IL-5 in Omenn's syndrome

Clin Exp Immunol. 1994 Jan;95(1):14-21. doi: 10.1111/j.1365-2249.1994.tb06008.x.

Abstract

Omenn's syndrome is a fatal, autosomal-recessive combined immune deficiency characterized by several erythematous exfoliative eruptions, lymphadenopathy, hepatosplenomegaly, and elevated eosinophil count. In some of these patients an expansion of CD3+CD4-CD8- double negative (DN) T cell population was observed. We show here that the DN population represents a clonal expansion of T cells which preferentially use V beta 14 in their T cell receptor complex. Using polymerase chain reaction, we show that patient's DN cells express spontaneously high levels of IL-5, thus possibly explaining the abundance of eosinophils in this disorder. The increase of IgE observed in patients with Omenn's syndrome is unlikely to be related to IL-4 production, as IL-4 levels in patient samples were low. However, patient's low expression of interferon-gamma (IFN-gamma), which has been reported to inhibit IgE production, may explain the elevated levels of IgE in this patient. The results thus highlight the importance of the inhibitory effect of IFN-gamma on regulation of IgE production.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antigens, CD / analysis*
  • CD3 Complex / analysis
  • CD4 Antigens / analysis
  • CD8 Antigens / analysis
  • Cytokines / analysis
  • Cytokines / genetics
  • Humans
  • Immunologic Deficiency Syndromes / immunology*
  • Infant
  • Interleukin-5 / analysis*
  • Male
  • RNA, Messenger / analysis
  • Receptors, Antigen, T-Cell, alpha-beta / analysis
  • T-Lymphocytes / chemistry
  • T-Lymphocytes / immunology*

Substances

  • Antigens, CD
  • CD3 Complex
  • CD4 Antigens
  • CD8 Antigens
  • Cytokines
  • Interleukin-5
  • RNA, Messenger
  • Receptors, Antigen, T-Cell, alpha-beta