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[遗传学与造血系统肿瘤]

[Genetics and hemopoietic neoplasia].

作者信息

Kurita S

出版信息

Gan To Kagaku Ryoho. 1982 May;9(5):774-81.

PMID:7184426
Abstract

Genetic factors suspected in the etiology of human hemopoietic neoplasia, such as leukemia, lymphoma and multiple myeloma, are reviewed. High incidence of consanguineous marriage was found in parents of familial leukemia in siblings. It was also noted that the age of patients with familial leukemia in children of consanguineous parents was younger than that of cases whose parents were not related. These findings suggest that genetic factors may play an important role in the etiology of familial leukemia in siblings. According to the frequencies of familial aggregations in close relatives, the genetic relationships were supposed to be important in chronic lymphocytic leukemia and acute leukemia, but not in chronic granulocytic leukemia. Increased prevalence of autoimmune diseases in relatives of leukemic patients suggests a possibility of genetic immunodeficiency as a common etiologic factor in both diseases. Immunodeficiency was found in unaffected relatives of patients with familial leukemia and lymphoma. Genetic factors were also suggested by the familial occurrences of multiple myeloma and primary macroglobulinemia, and the incidence of benign monoclonal gammopathy in relatives of patients with these diseases. HLA studies revealed the increased frequencies of A2 in acute lymphocytic leukemia, of B5 and B18 in Hodgkin's disease, and of A5 and B18 in multiple myeloma. From such relationships existing between familial immunodeficiencies and hemopoietic neoplasia, genes regulating the immune responsiveness might be involved in susceptibility to these diseases.

摘要

本文综述了疑似与人类造血系统肿瘤(如白血病、淋巴瘤和多发性骨髓瘤)病因相关的遗传因素。在家族性白血病患者的兄弟姐妹中,其父母近亲结婚的发生率较高。还注意到,近亲父母所生的家族性白血病患儿的发病年龄比父母无血缘关系的患儿要小。这些发现表明,遗传因素可能在家族性白血病的病因中起重要作用。根据近亲中家族聚集的频率,遗传关系在慢性淋巴细胞白血病和急性白血病中可能很重要,但在慢性粒细胞白血病中并非如此。白血病患者亲属中自身免疫性疾病患病率的增加表明,遗传免疫缺陷可能是这两种疾病的共同病因。在家族性白血病和淋巴瘤患者的未患病亲属中发现了免疫缺陷。多发性骨髓瘤和原发性巨球蛋白血症的家族聚集现象以及这些疾病患者亲属中良性单克隆丙种球蛋白病的发病率也提示了遗传因素。HLA研究显示,急性淋巴细胞白血病中A2频率增加,霍奇金病中B5和B18频率增加,多发性骨髓瘤中A5和B18频率增加。从家族性免疫缺陷与造血系统肿瘤之间存在的这种关系来看,调节免疫反应性的基因可能与这些疾病的易感性有关。

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