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重症联合型免疫缺陷(SCID)是具有遗传异质性的一组罕见的体液免疫和细胞免疫功能缺陷病,有X连锁或常染色体隐性遗传二种方式。SCID患者除了有典型的寿命缩短,还常发生恶性肿瘤。本文对常染色体隐性SCID基因携带者杂合子癌发病风险可能增高和这一假说是否成立进行验证。对象和方法:作者对1977年和1978年收集由全美免疫学专家提供的病例研究,包括ADA(腺苷脱氨酶)缺乏和Nezelof氏综合症在内的SCID患者的24个核心家系的5代系谱。所有与上述SCID患者的血
Severe combined immunodeficiency (SCID) is a group of rare humoral and cellular immunodeficiency diseases with genetic heterogeneity, with X-linked or autosomal recessive inheritance. In addition to SCID patients have a typical shortened life expectancy, but also often have malignant tumors. In this paper, autosomal recessive SCID carriers carriers of heterozygous cancer risk may be increased and the hypothesis is validated. Subjects and Methods: The authors collected case studies from national immunology experts in 1977 and 1978, and five generations of 24 nuclear pedigrees including SCID patients with ADA (adenosine deaminase) deficiency and Nezelof’s syndrome pedigree. All blood with SCID patients above