Study of the B cell memory compartment in common variable immunodeficiency

• Published in: European journal of immunology Vol. 30; no. 9; pp. 2516 - 2520
• Main Authors: Brouet, Jean‐Claude, Chedeville, Annick, Fermand, Jean‐Paul, Royer, Bruno
• Format: Journal Article
• Language: English
• Published: Weinheim WILEY‐VCH Verlag GmbH 01.09.2000
• Subjects: Antigens, CD; B-Lymphocytes - immunology; CD27 Ligand; CD27 / CD70; CD3 Complex - immunology; Cell Differentiation; Common variable immunodeficiency; Common Variable Immunodeficiency - immunology; Humans; Immunologic Memory; Lymphocyte Activation; Membrane Proteins - analysis; Memory B cell; T-Lymphocytes - immunology; TNF / TNF‐R; Tumor Necrosis Factor Receptor Superfamily, Member 7 - analysis; tumor necrosis factor receptors
• ISSN: 0014-2980; 1521-4141
• DOI: 10.1002/1521-4141(200009)30:9<2516::AID-IMMU2516>3.0.CO;2-Z

We evaluated the B cell memory pool among blood B cells from 20 patients with common variable immunodeficiency (CVID). CD27+ B cell number was normal or increased in 6 patients (with 95 % CD27+ B cells in 1 patient) and decreased in 14 patients. In 13 or 15 patients studied, the CD27 molecule was detectable on less than 50 % IgG or IgA B cells, indicating a defect in the maturation of these memory cells. Within the group of patients with a low number of CD27+ B cells, no up‐regulation of this molecule was observed after in vitro stimulation of purified B cells from 3 of 5 patients studied, suggesting an intrinsic B cell defect. In addition, ligation of the CD27 molecule was unable to trigger terminal differentiation of purified B cells in 1 of 2 cases with a large number of CD27+ B cells. Finally, the CD27 ligand was normally expressed on activated T cells in only 5 of 14 patients studied. These data confirm the heterogeneity of immunological defects in patients with CVID. Abnormal expression and / or function of the CD27‐CD70 members of the TNF / TNF receptor family contribute to the immunological defect.