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IgG2 Deficiency Associated with Defects in Production of Interferon‐Gamma; Comparison with Common Variable Immunodeficiency
Author(s) -
INOUE R.,
KONDO N.,
KOBAYASHI Y.,
FUKUTOMI O.,
ORII T.
Publication year - 1995
Publication title -
scandinavian journal of immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.934
H-Index - 88
eISSN - 1365-3083
pISSN - 0300-9475
DOI - 10.1111/j.1365-3083.1995.tb03544.x
Subject(s) - common variable immunodeficiency , interferon gamma , immunodeficiency , primary immunodeficiency , immunology , medicine , virology , biology , antibody , immune system
We report a novel mechanism of IgG2 deficiency. Several investigators have reported patients with IgG subclass deficiencies due to homozygous deletion of immunoglobulin heavy chain constant region genes. However, it is unclear what mechanism is responsible for IgG subclass deficiency in cases where no gene deletions have been detected and which are accompanied by recurrent infections due to aberrant immunoregulation. In the present study, we have focused our attention on production by peripheral blood mononuclear cells ( PBMCs ) of interferon‐gamma (IFN‐γ), which is known to induce IgG2 expression. PBMCs from four patients with IgG2 deficiency and their families were studied. Mitogen‐induced IFN‐γ production by PBMCs was decreased in all of the patients, although the proliferative responses of PBMCs and the percentages of CD3, CD4 , and CD8 T cell subsets were not decreased. IgG2 production by PBMCs was restored upon addition of IFN‐γ and mitogen to the PBMCs of the patients with IgG2 deficiency though it was not restored in the patients with common variable immunodeficiency. We conclude that defects in production of IFN‐γ play an important role in IgG2 deficiency.