Common variable immunodeficiency (CVID) and MxA‐protein expression in blood leucocytes

SUMMARY The underlying immunopathogenic mechanism of CVID has been suspected to involve a chronic viral infection or an autoimmune condition. However, formal proof of viral infection is lacking. Measurement of MxA‐protein in leucocyte lysates is a sensitive test for evaluating the activation of the...

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Published in:	Clinical and experimental immunology Vol. 101; no. 1; pp. 89 - 93
Main Authors:	RUMP, J. A., JAKSCHIESS, D., WALKER, U., SCHLESIER, M., WUSSOW, P., PETER, H. H.
Format:	Journal Article
Language:	English
Published:	Oxford, UK Blackwell Publishing Ltd 01-07-1995 Blackwell
Subjects:	Adult Aged AIDS/HIV Antigens, CD - biosynthesis Antigens, Differentiation, T-Lymphocyte - biosynthesis Antiviral Agents - physiology Biological and medical sciences CD4-CD8 Ratio CD57 Antigens CD8 Antigens - biosynthesis common variable immunodeficiency Common Variable Immunodeficiency - metabolism Female GTP-Binding Proteins Humans Immunodeficiencies. Immunoglobulinopathies Immunoglobulinopathies Immunopathology interferon‐alpha Leukocytes - chemistry Lymphocytes - metabolism Male Medical sciences Middle Aged MxA‐protein Myxovirus Resistance Proteins Protein Biosynthesis viral infection Human Immunopathology Common variable immunodeficiency Immunoglobulinopathy Leukocyte Etiopathogenesis Biological marker
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Summary:	SUMMARY The underlying immunopathogenic mechanism of CVID has been suspected to involve a chronic viral infection or an autoimmune condition. However, formal proof of viral infection is lacking. Measurement of MxA‐protein in leucocyte lysates is a sensitive test for evaluating the activation of the host's interferon system. Both viral infections and autoimmune diseases such as systemic lupus erythematosus (SLE) strongly induce MxA‐protein in peripheral leucocytes. We therefore examined 15 patients with longlasting hypogammaglobulinaemia for MxA‐protein induction in vivo: 13 patients suffered from CVID, one from hyper‐IgM syndrome, and one patient had chronic B lymphocytic leukaemia associated with immunoglobulin deficiency and chronic papilloma virus infection (condylomata accuminata). Only the latter patient exhibited a strong MxA‐protein expression; two CVID patients were borderline positive, and the remaining 12 patients including the hyper‐IgM syndrome were MxA‐protein‐negative. There was no relationship between MxA expression and low CD4/CD8 ratios or increased CD8/CD57+ T cell counts, although both conditions are often observed in CVID as well as in chronic viral infections. When exposed in vitro to interferon‐alpha (IFN‐α), peripheral blood leucocytes of four MxA‐negative patients were capable of producing normal amounts of MxA‐protein. Taken together, these results argue against a viral or autoimmune pathogenesis of CVID.
Bibliography:	ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-1 content type line 23 ObjectType-Article-1 ObjectType-Feature-2
ISSN:	0009-9104 1365-2249
DOI:	10.1111/j.1365-2249.1995.tb02282.x