| WEHR, Claudia, Teemu KIVIOJA, Christian SCHMITT, Berne FERRY, Torsten WITTE, Efrem EREN, Marcela VLKOVA, Manuel HERNANDEZ, Drahomíra DEDKOVA, Philip R. BOS, Gonke POERKSEN, Horst VON BEMUTH, Ulrich BAUMANN, Sigune GOLDACKER, Sylvia GUTENBERGER, Michael SCHLESIER, Florence BERGERON-VAN DER CRUYSSEN, Magali LE GARFF, Patrice DEBRÉ, Roland JACOBS, John JONES, Elizabeth BATERMAN, Jiří LITZMAN, P. Martin VAN HAGEN, Alessandro PLEBANI, Reinhold E. SCHMIDT, Vojtěch THON, Isabella QUINTI, Teresa ESPANOL, A. David WEBSTER, Helen CHAPEL, Mauno VIHINEN, Eric OKSENHENDLER, Hans Hartmut PETER and Klaus WARNATZ. The EUROclass trial: defining subgroups in common variable immunodeficiency. Blood. 2008, vol. 111, No 1, p. 77-85. ISSN 0006-4971. |
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@article{772619,
author = {Wehr, Claudia and Kivioja, Teemu and Schmitt, Christian and Ferry, Berne and Witte, Torsten and Eren, Efrem and Vlkova, Marcela and Hernandez, Manuel and Dedkova, Drahomíra and Bos, Philip R. and Poerksen, Gonke and von Bemuth, Horst and Baumann, Ulrich and Goldacker, Sigune and Gutenberger, Sylvia and Schlesier, Michael and Bergeronandvan der Cruyssen, Florence and Le Garff, Magali and Debré, Patrice and Jacobs, Roland and Jones, John and Baterman, Elizabeth and Litzman, Jiří and van Hagen, P. Martin and Plebani, Alessandro and Schmidt, Reinhold E. and Thon, Vojtěch and Quinti, Isabella and Espanol, Teresa and Webster, A. David and Chapel, Helen and Vihinen, Mauno and Oksenhendler, Eric and Peter, Hans Hartmut and Warnatz, Klaus},
article_number = {1},
keywords = {common variable immunodefciency; B-lymphocytes; classification},
language = {eng},
issn = {0006-4971},
journal = {Blood},
title = {The EUROclass trial: defining subgroups in common variable immunodeficiency.},
volume = {111},
year = {2008}
}
TY - JOUR
ID - 772619
AU - Wehr, Claudia - Kivioja, Teemu - Schmitt, Christian - Ferry, Berne - Witte, Torsten - Eren, Efrem - Vlkova, Marcela - Hernandez, Manuel - Dedkova, Drahomíra - Bos, Philip R. - Poerksen, Gonke - von Bemuth, Horst - Baumann, Ulrich - Goldacker, Sigune - Gutenberger, Sylvia - Schlesier, Michael - Bergeron-van der Cruyssen, Florence - Le Garff, Magali - Debré, Patrice - Jacobs, Roland - Jones, John - Baterman, Elizabeth - Litzman, Jiří - van Hagen, P. Martin - Plebani, Alessandro - Schmidt, Reinhold E. - Thon, Vojtěch - Quinti, Isabella - Espanol, Teresa - Webster, A. David - Chapel, Helen - Vihinen, Mauno - Oksenhendler, Eric - Peter, Hans Hartmut - Warnatz, Klaus
PY - 2008
TI - The EUROclass trial: defining subgroups in common variable immunodeficiency.
JF - Blood
VL - 111
IS - 1
SP - 77-85
EP - 77-85
SN - 00064971
KW - common variable immunodefciency
KW - B-lymphocytes
KW - classification
N2 - The heterogeneity of common variable immunodeficiency (CVID) calls for a classification addressing pathogenic mechanisms as well as clinical relevance. This European multicenter trial was initiated to develop a consensus of 2 existing classification schemes based on flowcytometric B-cell phenotyping and the clinical course. The clinical evaluation of 303 patients with the established diagnosis of CVID demonstrated a significant coincidence of granulomatous disease, autoimmune cytopenia, and splenomegaly. Phenotyping of B-cell subpopulations confirmed a severe reduction of switched memory B cells in most of the patients that was associated with a higher risk for splenomegaly and granulomatous disease. An expansion of CD21(low) B cells marked patients with splenomegaly. Lymphadenopathy was significantly linked with transitional B-cell expansion. Based on these findings and pathogenic consideration of B-cell differentiation, we suggest an improved classification for CVID (EUROclass), separating patients with nearly absent B cells (less than 1%), severely reduced switched memory B cells (less than 2%), and expansion of transitional (more than 9%) or CD21(low) B cells (more than 10%). Whereas the first group contains all patients with severe defects of early B-cell differentiation, severely reduced switched memory B cells indicate a defective germinal center development as found in inducible constimulator (ICOS) or CD40L deficiency. The underlying defects of expanded transitional or CD21(low) B cells remain to be elucidated.
ER -
WEHR, Claudia, Teemu KIVIOJA, Christian SCHMITT, Berne FERRY, Torsten WITTE, Efrem EREN, Marcela VLKOVA, Manuel HERNANDEZ, Drahomíra DEDKOVA, Philip R. BOS, Gonke POERKSEN, Horst VON BEMUTH, Ulrich BAUMANN, Sigune GOLDACKER, Sylvia GUTENBERGER, Michael SCHLESIER, Florence BERGERON-VAN DER CRUYSSEN, Magali LE GARFF, Patrice DEBRÉ, Roland JACOBS, John JONES, Elizabeth BATERMAN, Jiří LITZMAN, P. Martin VAN HAGEN, Alessandro PLEBANI, Reinhold E. SCHMIDT, Vojtěch THON, Isabella QUINTI, Teresa ESPANOL, A. David WEBSTER, Helen CHAPEL, Mauno VIHINEN, Eric OKSENHENDLER, Hans Hartmut PETER and Klaus WARNATZ. The EUROclass trial: defining subgroups in common variable immunodeficiency. \textit{Blood}. 2008, vol.~111, No~1, p.~77-85. ISSN~0006-4971.
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