Common Variable Immunodeficiency at the End of a Prospering Decade: Towards Novel Gene Defects and Beyond

Hermann Eibel; Ulrich Salzer; Klaus Warnatz

Disclosures

Curr Opin Allergy Clin Immunol. 2010;10(6):526-533. 

In This Article

IL21/IL21R

Analyzing responses of B and T cells from 32 CVID and 10 IgA-deficient (IgAD) patients to various combinations of anti-CD40, IL4, and IL21, Borte et al.[54] reported almost normal in-vitro formation of antibody secreting cells, class switch and IgG secretion for B cells from CVID patients, when IL21 was added to the cultures. Applying the same conditions, IgA secretion by B cells from IgAD patients was not restored to the extent as found for the CVID B cells. Genetic defects in the IL21 and IL21R genes were not found in the CVID patients.

The significance of the finding that IL21 may be a potent inducer of antibody production in CVID is to some extent limited due to the fact that 40% of the individuals were, according to the Freiburg classification, CVID subgroup II patients who have nearly normal switched memory B cells and who are known to often show normal B-cell responses in vitro.[54] Thus, it remains open whether IL21 can develop into a therapeutic option in CVID.

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