Reduced memory B cells in patients with hyper IgE syndrome

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 m...

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Veröffentlicht in:Clinical immunology (Orlando, Fla.). - 1999. - 129(2008), 3 vom: 17. Dez., Seite 448-54
1. Verfasser: Speckmann, C (VerfasserIn)
Weitere Verfasser: Enders, A, Woellner, C, Thiel, D, Rensing-Ehl, A, Schlesier, M, Rohr, J, Jakob, T, Oswald, E, Kopp, M V, Sanal, O, Litzman, J, Plebani, A, Pietrogrande, M C, Franco, J L, Espanol, T, Grimbacher, B, Ehl, S
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2008
Zugriff auf das übergeordnete Werk:Clinical immunology (Orlando, Fla.)
Schlagworte:Journal Article Multicenter Study Research Support, Non-U.S. Gov't Antibodies, Viral STAT3 Transcription Factor Immunoglobulin E 37341-29-0 DNA 9007-49-2
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520 |a 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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650 4 |a Multicenter Study 
650 4 |a Research Support, Non-U.S. Gov't 
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700 1 |a Enders, A  |e verfasserin  |4 aut 
700 1 |a Woellner, C  |e verfasserin  |4 aut 
700 1 |a Thiel, D  |e verfasserin  |4 aut 
700 1 |a Rensing-Ehl, A  |e verfasserin  |4 aut 
700 1 |a Schlesier, M  |e verfasserin  |4 aut 
700 1 |a Rohr, J  |e verfasserin  |4 aut 
700 1 |a Jakob, T  |e verfasserin  |4 aut 
700 1 |a Oswald, E  |e verfasserin  |4 aut 
700 1 |a Kopp, M V  |e verfasserin  |4 aut 
700 1 |a Sanal, O  |e verfasserin  |4 aut 
700 1 |a Litzman, J  |e verfasserin  |4 aut 
700 1 |a Plebani, A  |e verfasserin  |4 aut 
700 1 |a Pietrogrande, M C  |e verfasserin  |4 aut 
700 1 |a Franco, J L  |e verfasserin  |4 aut 
700 1 |a Espanol, T  |e verfasserin  |4 aut 
700 1 |a Grimbacher, B  |e verfasserin  |4 aut 
700 1 |a Ehl, S  |e verfasserin  |4 aut 
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