先天性免疫不全症候群の免疫学的・免疫血液学的研究
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概要
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An effort to clear the pathogenesis of the various types of immunological deficiency diseases was carried out, calling upon clinical characteristics, morphological description, genetic considerations and study of measurable functions of the immunologic apparatus. Comparison of these diseases with the animal rendered immunologically incompetent by thymectomy was also made. The cases under studies were four patients with congenital agammaglobulinemia (Bruton type), three with dysgammaglobulinemia Type 1, nine with ataxia telangiectasia and one with Wiskott-Aldrich syndrome. Congenital agammaglobulinemia (Bruton type), transmitted as a sex-linked recessive form, is characterized by marked decrease in IgG, absence of IgA, absence or gross deficiency of IgM and inability to form antibody against primary and secondary antigens. As a basis for this deficiency, these patients showed virtually a complete absence of plasma cells in their hematopoietic and lymphoid tissues. However, a small amount of IgG and IgM were demonstrated by fluorescent antibody technique at the site of the center of the lymph follicles. By contrast, these children had normal numbers of circulating lymphocytes and normal response to delayed hypersensitivity. Dysgammaglobulinemia Type 1 is characterized by an absence of IgG and IgA globulins in the presence of the normal level of IgM globulin in the serum. All these patients exhibited undue susceptibility to infection and lack of plasma cells in the hematopoietic and lymphoid tissues as was seen in children with congenital agammaglobulinemia. Ataxia telangiectasia is characterized by a progressive cerebellar ataxia, oculocutaneous telangiectasia and frequent sinopulmonary infections; it affects the sexes with equal frequency, and is probably transmitted as an autosomal recessive trait. Two cases of these patients had very low level of IgA globulin, but seven cases had normal level of IgA globulin in the serum. These patients had normal ability to produce circulating antibody, and normal numbers of plasma cells in the bone marrow and lymph node. All patients had low blood lymphocyte level less than 1,500 cells per mm^3. Their lymph nodes had been found to have poorly developed follicles and decreased numbers of lymphocytes. The in vitro reactivity to phytohemagglutinin of lymphocytes was reduced. There was almost complete failure of delayed hypersensitivity. Skin homograft was shown to have a distinctly prolonged survival more than 100 days and an abnormal process of rejection with very low infiltration of lymphoid cells. In mice (CF1) neonatally thymectomized, profoundly impaired development of the peripheral lymphoid tissue, as well as of the immune responsiveness including homograft rejection of the skin was observed. There are striking similarities in the immunological responsiveness of the patients with ataxia telangiectasia to those of laboratory animals thymectomized during neonatal life, and it is suggested that the immunological defect in ataxia telangiectasia may be primary in the thymus. Wiskott-Aldrich syndrome is characterized by a thrombocytopenia, eczema and recurrent infections. Serum IgM level was below normal value in presence of normal IgG and increased IgA globulin. This case of Wiskott-Aldrich syndrome was able to respond with antibody synthesis to immunization with typhoid-Paratyphoid vaccine, but had only small amount of natural isoagglutinin. Tuberculin reaction remained negative after BCG inoculation while the skin homograft was rejected in normal manner. In conclusion, these findings stated above seem to support the hypothesis of Good that those immunological deficiency diseases may be classified into two categories, i.e., thymus dependent and Bursa equivalent lymphoid tissue dependent.
- 1969-11-30