| Research Abstract |
Ten Epstein-Barr virus (EBV)-immoratalized cell lines from patients with primary immunodefiicency including ataxia telangiectasia (AT), Wiskott-Aldrich syndrome (WAS), severe combined immunodeficiency (SCID) and common variable immunodeficiency (CVID) were investigated regarding growth capability both in fresh medium and soft agarose, the expression of EBV-related latent antigens (EB V-nuclear antigen [EBNA]-1,-2,-3a,-3b,-3c,leder protein [LP]. and latent membrane protein [LMP]-1,-2a and 2b), chromosomal aberrations, the expression of several oncogenes and tumor suppressor genes, the expression of NF-κB, the effect of anti-B cell monoclonal antibodies to the growth, the expression of a couple of cytokines, and degree of EB V-specific cytotoxic T lymphocytes (EBV-CTL) and NK cell activities with or without use of cyclosporine A (CSA), which were compared to those from healthy counterparts. Cells from patients with primary immunodeficiency expressed all EB V-related latent antigens. EB V
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-CTL and NK cell activities were significantly decreased in almost patients, associated with decreased expression and/or production of interferon-γ. Use of anti-B cell monoclonal antibodies resulted in decreased growth capacity corresponding to its concentration. Some cells from patients with AT showed high saturation density and increased colony forming efficiency associated with increased expression of myc and ras family with some chromosomal aberrations.
These results suggested defect of immunosurveillance such as lack of EB V-CTL and/or NK cell activities are highly responsible for the development of EB V-LPD in patients with primary immunodeficiency. However, some cells form AT, one of chromosome breakage syndrome, have a tendency to have more malignant phenotype even in the early phase of cultivation. Therefore, use of anti-B cell monoclonal antibodies may be beneficial for EB V-LPD in patients with primary immunodeficiency, and early diagnosis and treatment are highly recommended especially in patients with chromosomal instability and profound immunodeficiency. Less
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