关于沃纳综合征的免疫学研究。
Immunologic studies on Werner's syndrome.
作者信息
Nakao Y, Hattori T, Takatsuki K, Kuroda Y, Nakaji T, Fujiwara Y, Kishihara M, Baba Y, Fujita T
出版信息
Clin Exp Immunol. 1980 Oct;42(1):10-9.
Five patients with Werner's syndrome were studied to elucidate the relationship between the immune system and the ageing process. Three of five patients had neoplasms such as fibrosarcoma, meningioma and thyroid adenoma but none of the five patients manifested symptoms and signs suggestive of typical autoimmune disorders. Most immunohaematological data were normal; however, two patients exhibited diminished lymphocyte functions such as a significant (P less than 0 . 05) reduction in pokeweed mitogen (PWM) induced B cell differentiation, a slightly decreased response to Con A- or mitomycin-treated allogeneic lymphocytes and naturally occurring serum antibody to T cells. While these immunological changes were quantitatively small, subnormal levels of immune functions were detected. Cultured fibroblasts exhibited a decrease in clonal growth potentials and elongation of newly synthesized DNA, a decreased expression of HLA antigens, and a diminished secretion of beta 2-microglobulin.
对五名患有沃纳综合征的患者进行了研究，以阐明免疫系统与衰老过程之间的关系。五名患者中有三名患有纤维肉瘤、脑膜瘤和甲状腺腺瘤等肿瘤，但五名患者均未表现出提示典型自身免疫性疾病的症状和体征。大多数免疫血液学数据正常；然而，两名患者表现出淋巴细胞功能减退，如商陆有丝分裂原（PWM）诱导的B细胞分化显著降低（P小于0.05），对刀豆蛋白A或丝裂霉素处理的同种异体淋巴细胞的反应略有降低，以及天然存在的抗T细胞血清抗体。虽然这些免疫变化在数量上较小，但检测到免疫功能低于正常水平。培养的成纤维细胞表现出克隆生长潜能降低、新合成DNA延长、HLA抗原表达降低以及β2-微球蛋白分泌减少。
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