Expansion of T helper type 17 lymphocytes in patients with chronic granulomatous disease

Clin Exp Immunol. 2011 Oct;166(1):26-33. doi: 10.1111/j.1365-2249.2011.04449.x.

Abstract

Hyper-immunoglobulin (Ig)E syndrome (HIES) is a primary immunodeficiency associated with mutations in STAT3 resulting in impaired development of T helper type 17 (Th17) lymphocytes. HIES patients with a reduced frequency of Th17 cells present with infections caused by Staphylococcus aureus and/or Candida strains. The same spectrum of pathogens is present in patients with chronic granulomatous disease (CGD).We analysed the characteristics of the Th17 compartment in HIES and CGD. HIES patients showed very low numbers of Th17 cells. By contrast, the frequency of Th17 cells and production of Th17-derived cytokines was significantly higher among CGD patients when compared to both control samples and HIES. Naive CD4(+) cells in CGD patients had a normal capacity to differentiate into IL-17-producing cells and the numbers of Th17 cells in the CGD patients normalized following successful bone marrow transplantation. Our findings complement recent data on the importance of Th17 cells for elimination of infections with C. albicans and S. aureus.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Candida albicans / growth & development
  • Candidiasis / immunology
  • Candidiasis / microbiology
  • Case-Control Studies
  • Cell Differentiation / immunology
  • Child
  • Child, Preschool
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Flow Cytometry
  • Granulomatous Disease, Chronic / genetics
  • Granulomatous Disease, Chronic / immunology*
  • Granulomatous Disease, Chronic / metabolism
  • Granulomatous Disease, Chronic / pathology
  • Granulomatous Disease, Chronic / therapy
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Interferon-gamma / immunology*
  • Interferon-gamma / metabolism
  • Interleukin-17 / immunology*
  • Interleukin-17 / metabolism
  • Job Syndrome / genetics
  • Job Syndrome / immunology*
  • Job Syndrome / metabolism
  • Job Syndrome / pathology
  • Lymphocyte Count
  • Male
  • Membrane Glycoproteins / genetics
  • Membrane Glycoproteins / immunology*
  • Membrane Glycoproteins / metabolism
  • Mutation
  • NADPH Oxidase 2
  • NADPH Oxidases / genetics
  • NADPH Oxidases / immunology*
  • NADPH Oxidases / metabolism
  • STAT3 Transcription Factor / genetics
  • STAT3 Transcription Factor / immunology*
  • STAT3 Transcription Factor / metabolism
  • Staphylococcal Infections / immunology
  • Staphylococcal Infections / microbiology
  • Staphylococcus aureus / growth & development
  • Th17 Cells / immunology*
  • Th17 Cells / metabolism
  • Th17 Cells / pathology
  • Transplantation, Homologous

Substances

  • Interleukin-17
  • Membrane Glycoproteins
  • STAT3 Transcription Factor
  • Interferon-gamma
  • CYBB protein, human
  • NADPH Oxidase 2
  • NADPH Oxidases