Frequency of Treg cells is reduced in CVID patients with autoimmunity and splenomegaly and is associated with expanded CD21lo B lymphocytes

J Clin Immunol. 2010 Mar;30(2):292-300. doi: 10.1007/s10875-009-9351-3. Epub 2009 Dec 9.

Abstract

Introduction: Common variable immunodeficiency is a heterogeneous antibody deficiency syndrome with autoimmune and inflammatory complications in a significant proportion of patients. The study was designed to evaluate the role of T regulatory (Treg) cells in common variable immunodeficiency (CVID) patients with autoimmunity.

Methods: The number and frequency of Treg cells (CD4(+), CD25(hi), Foxp3(+)) were evaluated in patients and controls, and Foxp3 expression in different subgroups of CVID patients with common clinical manifestations was compared.

Results: CVID patients had significantly fewer Treg cells than controls, and low frequency of Treg cells was associated with expansion of CD21(lo) B cells in patients. Patients with autoimmunity had significantly reduced frequency but normal numbers of regulatory T cells, whilst patients with splenomegaly had significant reduction in frequency and number of regulatory T cells.

Conclusion: Foxp3 is useful on its own or as an adjunct to classify CVID patients although the possibility of reduction in Treg cells as a secondary phenomenon cannot be excluded.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antigens, CD / biosynthesis
  • Autoimmunity
  • B-Lymphocytes / immunology
  • B-Lymphocytes / metabolism*
  • B-Lymphocytes / pathology
  • Cell Proliferation
  • Cell Separation
  • Cells, Cultured
  • Common Variable Immunodeficiency / immunology*
  • Common Variable Immunodeficiency / pathology
  • Common Variable Immunodeficiency / physiopathology*
  • Female
  • Flow Cytometry
  • Forkhead Transcription Factors / biosynthesis
  • Humans
  • Male
  • Middle Aged
  • Splenomegaly
  • T-Lymphocytes, Regulatory / immunology
  • T-Lymphocytes, Regulatory / metabolism*
  • T-Lymphocytes, Regulatory / pathology

Substances

  • Antigens, CD
  • FOXP3 protein, human
  • Forkhead Transcription Factors