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. V. Kopp; O. Sanal; J. Litzman; A. Plebani; M. C. Pietrogrande; J. L. Franco; T. Espanol; B. Grimbacher; S. Ehl

doi:10.1016/j.clim.2008.08.002

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. V. Kopp, O. Sanal, J. Litzman, A. Plebani, M. C. Pietrogrande, J. L. Franco, T. Espanol, B. Grimbacher, S. Ehl^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

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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.

Original language	English
Pages (from-to)	448-454
Number of pages	7
Journal	Clinical Immunology
Volume	129
Issue number	3
DOIs	https://doi.org/10.1016/j.clim.2008.08.002
Publication status	Published - Dec 2008
Externally published	Yes

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10.1016/j.clim.2008.08.002

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Speckmann, C., 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. (2008). Reduced memory B cells in patients with hyper IgE syndrome. Clinical Immunology, 129(3), 448-454. https://doi.org/10.1016/j.clim.2008.08.002

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title = "Reduced memory B cells in patients with hyper IgE syndrome",

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.",

keywords = "Atopic dermatitis, B cells, Hyper IgE syndrome, Memory cells, STAT-3",

author = "C. Speckmann and A. Enders and C. Woellner and D. Thiel and A. Rensing-Ehl and M. Schlesier and J. Rohr and T. Jakob and E. Oswald and Kopp, {M. V.} and O. Sanal and J. Litzman and A. Plebani and Pietrogrande, {M. C.} and Franco, {J. L.} and T. Espanol and B. Grimbacher and S. Ehl",

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doi = "10.1016/j.clim.2008.08.002",

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Speckmann, C, Enders, A, Woellner, C, Thiel, D, Rensing-Ehl, A, Schlesier, M, Rohr, J, Jakob, T, Oswald, E, Kopp, MV, Sanal, O, Litzman, J, Plebani, A, Pietrogrande, MC, Franco, JL, Espanol, T, Grimbacher, B & Ehl, S 2008, 'Reduced memory B cells in patients with hyper IgE syndrome', Clinical Immunology, vol. 129, no. 3, pp. 448-454. https://doi.org/10.1016/j.clim.2008.08.002

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AU - Speckmann, C.

AU - Enders, A.

AU - Woellner, C.

AU - Thiel, D.

AU - Rensing-Ehl, A.

AU - Schlesier, M.

AU - Rohr, J.

AU - Jakob, T.

AU - Oswald, E.

AU - Kopp, M. V.

AU - Sanal, O.

AU - Litzman, J.

AU - Plebani, A.

AU - Pietrogrande, M. C.

AU - Franco, J. L.

AU - Espanol, T.

AU - Grimbacher, B.

AU - Ehl, S.

PY - 2008/12

Y1 - 2008/12

N2 - 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.

AB - 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.

KW - Atopic dermatitis

KW - B cells

KW - Hyper IgE syndrome

KW - Memory cells

KW - STAT-3

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DO - 10.1016/j.clim.2008.08.002

M3 - Article

SN - 1521-6616

VL - 129

SP - 448

EP - 454

JO - Clinical Immunology

JF - Clinical Immunology

IS - 3

ER -

Reduced memory B cells in patients with hyper IgE syndrome

Abstract

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