Nijmegen breakage syndrome: Long-term monitoring of viral and immunological biomarkers in peripheral blood before development of malignancy

Abstract Selected viruses and immune parameters were monitored in 57 patients with Nijmegen breakage syndrome as a proposed tool for early detection of changes preceding development of malignancy. The following parameters were analysed: (1) viral infections; (2) monoclonal proteins; and (3) B-cell a...

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Published in:Clinical immunology (Orlando, Fla.) Fla.), 2010-06, Vol.135 (3), p.440-447
Main Authors: Gregorek, Hanna, Chrzanowska, Krystyna H, Dzierżanowska-Fangrat, Katarzyna, Wakulińska, Anna, Pietrucha, Barbara, Zapaśnik, Anna, Zborowska, Magdalena, Pac, Małgorzata, Smółka-Afifi, Dominika, Kasztelewicz, Beata, Piekutowska-Abramczuk, Dorota, Małdyk, Jadwiga
Format: Article
Language:English
Subjects:
Adolescent
Allergy and Immunology
Biological and medical sciences
Biomarkers, Tumor - analysis
Child
Child, Preschool
Chromosome fragility (bloom syndrome, ataxia telangiectasia, fanconi anemia, x-linked mental retardation...)
Epstein-Barr virus
Female
Fundamental and applied biological sciences. Psychology
Fundamental immunology
Gene Rearrangement, B-Lymphocyte
Gene Rearrangement, T-Lymphocyte
Hematologic Neoplasms - epidemiology
Hematologic Neoplasms - etiology
Humans
Ig/TCR chain gene rearrangements
Immunodeficiencies. Immunoglobulinopathies
Immunoglobulinopathies
Immunoglobulins
Immunopathology
Infant
Infectious diseases
Male
Malignancy
Medical genetics
Medical sciences
Monoclonal gammopathy
Nijmegen breakage syndrome
Nijmegen Breakage Syndrome - blood
Nijmegen Breakage Syndrome - immunology
Nijmegen Breakage Syndrome - virology
Paraproteinemias
Receptors, Antigen, B-Cell - genetics
Receptors, Antigen, T-Cell - genetics
Reverse Transcriptase Polymerase Chain Reaction
T-Lymphocyte Subsets - immunology
Viral diseases
Viral infections
Virus Diseases - complications
Virus Diseases - epidemiology
Young Adult
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Summary:Abstract Selected viruses and immune parameters were monitored in 57 patients with Nijmegen breakage syndrome as a proposed tool for early detection of changes preceding development of malignancy. The following parameters were analysed: (1) viral infections; (2) monoclonal proteins; and (3) B-cell and T-cell receptor gene rearrangements in peripheral blood lymphocytes. Viral infections were detected in 68.4% of patients with a predominance of EBV (63.2%), followed by HBV (19.2%) and HCV (8.8%). Monoclonal gammopathy detected in 38.6% of cases correlated with the presence of EBV DNA ( p = 0.002) and HCV RNA ( p = 0.04). Clonal Ig and/or TCR gene rearrangements occurred in 73.9% of patients. The presence of at least one of the studied parameters preceded the development of malignancy in 22 patients. Systematic PCR analysis for viral infections and Ig/TCR gene rearrangements, supplemented by detection of monoclonal proteins, is advantageous in monitoring NBS patients before severe complications of the disease, including cancer, appear.
ISSN:1521-6616
1521-7035
DOI:10.1016/j.clim.2010.01.008