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.
English
Settore MED/38 - Pediatria Generale e Specialistica
Articolo
Sì, ma tipo non specificato
2008
Academic Press
129
3
448
454
Periodico con rilevanza internazionale
info:eu-repo/semantics/article
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]
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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,...espandi
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/51203
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