Journal of Clinical Immunology

, Volume 23, Issue 5, pp 385–400

Common Variable Immunodeficiency Patient Classification Based on Impaired B Cell Memory Differentiation Correlates with Clinical Aspects

  • B. Piqueras
  • C. Lavenu-Bombled
  • L. Galicier
  • F.Bergeron-Van Der Cruyssen
  • L. Mouthon
  • S. Chevret
  • P. Debré
  • C. Schmitt
  • E. Oksenhendler
Article

Abstract

Common variable immunodeficiency (CVID) is a very heterogeneous syndrome defined by impaired immunoglobulin production. The functional classification of CVID patients on the basis of in vitro immunoglobulin production is time consuming and has never shown any predictive value. We propose a classification based on the quantitative repartition of naive/memory B cells according to the dual expression of IgD and CD27. Fifty-seven patients were categorized into three groups: Group MB2 (11 patients, 19%) with normal memory B cells; Group MB1 (19 patients, 33%) with defective switched memory (IgDCD27+) but normal nonswitched memory B cells (IgD+CD27+); Group MB0 (27 patients, 47%) with almost no memory B cells. In addition, a downexpression of activation markers (CD25, CD21, CD80, CD86) on B cells characterized the group MB1 patients and was associated with an upexpression of activation markers (HLA-DR, CD95, CD57) on T cells. This classification correlates with some clinical aspects showing a higher prevalence of splenomegaly (16/27, 59%), lymphoid proliferation (13/27, 48%) and granulomatous disease (12/27, 44%) in group MB0. Splenomegaly was also frequent in group MB1 (8/19, 42%). In contrast, autoimmunity was observed with similar prevalence in all three groups. Moreover, by analyzing B cell phenotype, immunoglobulin transcript expression, and somatic mutations, we propose different putative mechanisms responsible for impaired B cell activation and memory differentiation in this syndrome.

CVID classification CD27 B cell memory 

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Copyright information

© Plenum Publishing Corporation 2003

Authors and Affiliations

  • B. Piqueras
    • 1
  • C. Lavenu-Bombled
    • 1
  • L. Galicier
    • 2
  • F.Bergeron-Van Der Cruyssen
    • 1
  • L. Mouthon
    • 3
  • S. Chevret
    • 4
  • P. Debré
  • C. Schmitt
    • 1
  • E. Oksenhendler
    • 2
  1. 1.Laboratoire d'Immunologie Cellulaire, INSERM U543Hôpital Pitié-SalpétrièreParisFrance
  2. 2.Service d'Immunpathologie CliniqueHôpital St. Louis, AP-HPParisFrance
  3. 3.Service de Médecine InterneHôpital Avicenne, AP-HPBobignyFrance
  4. 4.Département de Biostatistique MédicaleHôpital St. Louis, AP-HP, cParisFrance

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