Reduced memory B cells in patients with hyper IgE syndrome

Clin Immunol. 2008 Dec;129(3):448-54. doi: 10.1016/j.clim.2008.08.002. Epub 2008 Oct 2.


Dominant-negative mutations in STAT-3 have recently been found in the majority of patients with sporadic or autosomal-dominant hyper IgE syndrome (HIES). Since STAT-3 plays a role in B cell development and differentiation, we analyzed memory B cells in 20 patients with HIES, 17 of which had STAT-3 mutations. All but four patients had reduced non-switched and/or class-switched memory B cells. No reduction in these B cell populations was found in 16 atopic dermatitis patients with IgE levels above 1000 KU/L. There was no correlation between the reduction of memory B cells and the ability to produce specific antibodies. Moreover, there was no correlation between the percentage of memory B cells and the infection history. Analysis of memory B cells can be useful in distinguishing patients with suspected HIES from patients with atopic disease, but probably fails to identify patients who are at high risk of infection.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Antibodies, Viral / blood
  • Antibody Formation
  • B-Lymphocytes / immunology*
  • B-Lymphocytes / pathology
  • Child
  • Cohort Studies
  • DNA / chemistry
  • DNA / genetics
  • Dermatitis, Atopic / genetics
  • Dermatitis, Atopic / immunology
  • Dermatitis, Atopic / pathology
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Flow Cytometry
  • Genotype
  • Humans
  • Immunoglobulin E / immunology
  • Immunoglobulin E / metabolism
  • Immunologic Memory / genetics
  • Immunologic Memory / immunology*
  • Job Syndrome / genetics
  • Job Syndrome / immunology*
  • Job Syndrome / pathology
  • Male
  • Middle Aged
  • Mutation
  • STAT3 Transcription Factor / genetics
  • STAT3 Transcription Factor / immunology
  • Young Adult


  • Antibodies, Viral
  • STAT3 Transcription Factor
  • Immunoglobulin E
  • DNA