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The EUROclass trial: defining subgroups in common variable immunodeficiency.

In English

Författare

  • Claudia Wehr
  • Teemu Kivioja
  • Christian Schmitt
  • Berne Ferry
  • Torsten Witte
  • Efrem Eren
  • Marcela Vlkova
  • Manuel Hernandez
  • Drahomira Detkova
  • Philip R Bos
  • Gonke Poerksen
  • Horst von Bernuth
  • Ulrich Baumann
  • Sigune Goldacker
  • Sylvia Gutenberger
  • Michael Schlesier
  • Florence Bergeron-van der Cruyssen
  • Magali Le Garff
  • Patrice Debré
  • Roland Jacobs
  • John Jones
  • Elizabeth Bateman
  • Jiri Litzman
  • P Martin van Hagen
  • Alessandro Plebani
  • Reinhold E Schmidt
  • Vojtech Thon
  • Isabella Quinti
  • Teresa Espanol
  • A David Webster
  • Helen Chapel
  • Mauno Vihinen
  • Eric Oksenhendler
  • Hans Hartmut Peter
  • Klaus Warnatz
Visa allt

Summary, in English

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. This trial is re-gistered at http://www.uniklinik-freiburg.de/zks/live/uklregister/Oeffentlich.html as UKF000308.

Publiceringsår

2008

Språk

Engelska

Sidor

77-85

Publikation/Tidskrift/Serie

Blood

Volym

111

Issue

1

Dokumenttyp

Artikel i tidskrift

Förlag

American Society of Hematology

Ämne

  • Hematology

Nyckelord

  • B-Lymphocytes: immunology
  • B-Lymphocytes: pathology
  • Common Variable Immunodeficiency: classification
  • Common Variable Immunodeficiency: epidemiology
  • Common Variable Immunodeficiency: immunology
  • Common Variable Immunodeficiency: pathology
  • Europe: epidemiology
  • Homeostasis: immunology
  • Immunoglobulins: blood

Status

Published

ISBN/ISSN/Övrigt

  • ISSN: 1528-0020