書誌事項
- タイトル別名
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- Ataxia telangiectasia and characterization of it's immunological disorders.
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抄録
Ataxia telangiectasia (AT) is a primary immunodeficiency syndrome characteri zed by cerebellar ataxia, extrapyramidal signs, oculocutaneous telangiectasia, recurrent respiratory infections and development of malignancies. AT is a complex autosomal recessive disorder involving several systems other than lymphoid cells or the central nervous system. Such a diversity of abnormalities includes hypersensitivity of fibroblasts and lymphocytes to ionizing radiation (anomaly of DNA repair), non-random chromosomal rearrangements in lymphocytes, elevated serum level of α-fetoprotein, premature aging and endocrine disorders. A DNA processing or repair protein is the suspected common denominator in this pathology. Whatever the putative common underlying mechanism, AT patients have profound alterations of the humoral and cellular immune system whose mechanisms should be discussed in terms similar to those for other immunodeficiency diseases.<BR>The usual immunological abnormalities in this disease include decreased levels of CD 3 and CD 4 positive T lymphocytes, impaired delayed hypersensitivity, hypoplasia of thymus, decreased blast transformation in vitro in response to mitogen or antigenic stimulation, and decreased levels of serum IgA, IgE, and IgG 2 subclass.<BR>In this paper, the results of our recent studies on the defects of B cells in patients with AT were presented. (1) We found that the geometric means of IgA production in the supernatants of the lymphoblastoid cell lines established by EB virus, from all patients with AT, were significantly lower than those from healthy controls (P<0.01). (2) IgG subclasses of the patients' sera were also measured by ELISA, and IgG 4 was defective in four cases among six patients with AT. Both results that indicate a defect of humoral immunity in patients with AT is attributable in part to an intrinsic abnormality of B cells. (3) Eleven patients with AT were studied concerning the status of specific EBV serology. All the AT patients had high EBV antibody titers of IgG to viral capsid antigen (VCA) and early antigen (EA), and low titers of IgG to EBV- associated nuclear antigen (EBNA) compared with healthy controls. It is considered to be related with the deficiency of EBV specific T cell killer function. (4) In the study of reactivity of peripheral lymphocytes to EB virus in the patients with AT, lymphoblastoid cell lines from the patients easily expressed EA and VCA by P 3 HR-1 strain EBV infection. These results suggest that a defect in immune surveillance and target cell defectiveness with genetic predisposition are the major factors of virus induced carcinogenesis.
収録刊行物
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- 脳と発達
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脳と発達 22 (2), 103-111, 1990
THE JAPANESE SOCIETY OF CHILD NEUROLOGY