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早在1974年,作者观察到一种联合免疫缺陷病伴有细胞的HLA—A和B抗原表达缺如的疾病,称之为无标记淋巴细胞综合征(BLS)。不久,又发现一个土耳其人家族中的2例患者罹患类似综合征。所有这些病例的免疫缺陷病均伴随淋巴细胞表面HLA-A、B和C抗原以及β_2微球蛋白缺如。这种BLS除了提供免疫缺陷病的另一种病因线索外,还可提出关于MHC决定簇在淋巴细胞分化和功能中的作用的重要资料。由于本综合征具有重要的基本意义,因此,世界卫生组织在法国里昂设立了国际登记处,现将所报告的9例患者摘要报道如下。
As early as 1974, the authors observed a deficiency of HLA-A and B antigen-associated diseases in co-immunodeficiency disease-associated cells called unlabelled lymphocyte syndrome (BLS). Shortly thereafter, two more patients in a Turkish family were found to have similar syndromes. Immunodeficiency in all these cases is accompanied by a lack of HLA-A, B and C antigens on the surface of lymphocytes and absence of β-2 microglobulin. In addition to providing another etiological clue to immunodeficiency disease, such BLS may also provide important information on the role of MHC determinants in lymphocyte differentiation and function. As this syndrome has important basic meaning, WHO has set up an international registry in Lyon, France. The summary of the 9 reported patients is now reported as follows.