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

• 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
• DOI: 10.1111/j.1365-2249.1995.tb02282.x
• EISSN: 1365-2249
• ISSN: 0009-9104

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.