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Reduced memory B cells in patients with hyper IgE syndrome
C Speckmann1, A Enders, C Woellner
1Center for Pediatrics and Adolescent Medicine, University of Freiburg, Mathildenstr 1, 79106 Freiburg, Germany.
Clinical Immunology (Orlando, Fla.)
|October 7, 2008
Summary
Dominant-negative STAT-3 mutations are common in Hyper IgE Syndrome (HIES). Patients with HIES often show reduced memory B cells, distinguishing them from atopic dermatitis patients.
Area of Science:
- Immunology
- Genetics
Background:
- Signal transducer and activator of transcription 3 (STAT-3) is crucial for B cell development.
- Dominant-negative STAT-3 mutations are implicated in Hyper IgE Syndrome (HIES).
Purpose of the Study:
- To investigate memory B cell populations in patients with HIES.
- To differentiate HIES patients from those with atopic dermatitis based on B cell analysis.
Main Methods:
- Analysis of memory B cells in 20 HIES patients (17 with STAT-3 mutations) and 16 atopic dermatitis patients.
- Comparison of B cell populations and correlation with antibody production and infection history.
Main Results:
- Reduced non-switched and/or class-switched memory B cells were observed in most HIES patients.
- No similar reduction was found in atopic dermatitis patients.
- No correlation was found between memory B cell reduction and antibody production or infection history.
Conclusions:
- Memory B cell analysis can help distinguish HIES from atopic disease.
- Memory B cell analysis may not predict infection risk in HIES patients.
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