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CD4+ cells from patients with Common Variable Immunodeficiency have a reduced ability of CD40 ligand membrane expression after in vitro stimulation
Author(s) -
Brugi Duilio,
Airò Paolo,
Lebovitz Morena,
Malacarne Fabio,
Ugazio Alberto G.,
Notarangelo Luigi D.,
Plebani Alessandro,
Cattaneo Roberto
Publication year - 1996
Publication title -
pediatric allergy and immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.269
H-Index - 89
eISSN - 1399-3038
pISSN - 0905-6157
DOI - 10.1111/j.1399-3038.1996.tb00129.x
Subject(s) - common variable immunodeficiency , cd40 , ionomycin , stimulation , medicine , immunology , immunodeficiency , antibody , in vitro , immune system , biology , genetics , cytotoxic t cell
Background: Common Variable Immunodeficiency (CVID) is characterized by defective antibody production. This has been variably attributed to intrinsic B‐cell defects or to T‐cell disfunctions. Recently, it has been reported that the expression of the CD40 Ligand (CD40L), a T‐cell surface molecule that plays a critical role in the cell‐contact‐mediated helper signals provided to B‐cells, is defective in a subset of patients with CVID. Methods: To demonstrate that the defective expression is due to intrinsic functional abnormalities of CD4+ lymphocytes, CD4+ cells were purified from eight patients with CVID and eight age‐paired controls, stimulated with PMA+Ionomycin. and studied for CD40L expression by flow cytome‐try using specific monoclonal antibodies. Results and conclusions: The percentage of CD4+ cells expressing CD40L after optimal stimulation was correlated with age both in patients with CVID (r: 0. 74: p: 0.04) and in healthy controls (r: 0. 73; p: 0.04). The percentage of CD40L+ cells was reduced in patients with CVID compared to that of controls (p: 0.02 when data are paired for age) with a reduced density of expression (p: < 0.01). The defect was variable in different patients and in some cases it was marginal.