B cells from common variable immunodeficiency patients fail to differentiate to antibody secreting cells in response to TLR9 ligand (CpG-ODN) or anti-CD40+IL21 |
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Authors: | Clemente Antonio Pons Jaime Matamoros Nuria Iglesias Julio Ferrer Joana M |
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Institution: | Servei d’Immunologia, Institut Universitari d’Investigació en Ciències de la Salut (IUNICS), Hospital Son Dureta, Palma de Mallorca, Spain |
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Abstract: | Common variable immunodeficiency (CVID) is a primary immunodeficiency characterised by hypogammaglobulinaemia and antibody deficiency to T dependent and independent antigens. Patients suffer from recurrent respiratory infections and poor response to vaccination. Although the underlying molecular defect is unknown, most CVID patients show impaired late B cell differentiation. We investigated B cell differentiation and immunoglobulin secretion induced by two different stimuli: TLR9 specific ligand (CpG-ODN) and anti-CD40 combined with IL21. The contribution of BCR signalling (anti-IgM stimulation) was also evaluated. B cells from CVID patients produced low levels of IgG and IgA in response to both kinds of stimuli that was not restored by anti-IgM. Production of IgM was conserved when cells were stimulated with anti-CD40 and IL21. These results point to a wide signalling defect in B lymphocytes from CVID patients that may be related to their hypogammaglobulinaemia and poor response to vaccination. |
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Keywords: | Common variable immunodeficiency B lymphocytes TLRs CpG-ODN IL21 |
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