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.
Reduced memory B cells in patients with hyper IgE syndrome / C. Speckmann, A. Enders, C. Woellner, D. Thiel, A. Rensing-Ehl, M. Schlesier, J. Rohr, T. Jakob, E. Oswald, M. Kopp, O. Sanal, J. Litzman, A. Plebani, M.C. Pietrogrande, J. Franco, T. Espanol, B. Grimbacher, S. Ehl. - In: CLINICAL IMMUNOLOGY. - ISSN 1521-6616. - 129:3(2008), pp. 448-454. [10.1016/j.clim.2008.08.002]
Reduced memory B cells in patients with hyper IgE syndrome
M.C. Pietrogrande;
2008
Abstract
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.
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