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家族性多发性骨髓瘤。对37个家族的综述。

Familial multiple myeloma. A review of thirty-seven families.

作者信息

Shoenfeld Y, Berliner S, Shaklai M, Gallant L A, Pinkhas J

出版信息

Postgrad Med J. 1982 Jan;58(675):12-6. doi: 10.1136/pgmj.58.675.12.

Abstract

The review of the pertinent literature disclosed 36 reports of familial multiple myeloma, described mostly in siblings, to which the authors add one more family. These patients did not differ significantly from those with non-familial myeloma with regard to sex, age, distribution of monoclonal proteins, clinical and laboratory data, and the course and prognosis of the disease. An increased incidence of immunoglobulin abnormalities was observed in healthy relatives of patients affected with familial myeloma. In most cases the time interval of the diagnosis of myeloma in a family member of a known patient was under 4 years. These observations, in conjunction with reports of myeloma occurring in clusters in a community and the appearance of myeloma in spouses raise the possibility of an environmental factor (virus?) which may contribute to the pathogenesis of myeloma in genetically predisposed individuals. Multiple myeloma should be added to the list of neoplastic diseases in which the family history is relevant and in which genetic and possibly environmental factors may be pathogenetically involved.

摘要

对相关文献的回顾发现了36例家族性多发性骨髓瘤的报告,大多描述的是兄弟姐妹中的病例,作者在此基础上又增加了一个家族。这些患者在性别、年龄、单克隆蛋白分布、临床和实验室数据以及疾病进程和预后方面与非家族性骨髓瘤患者并无显著差异。在家族性骨髓瘤患者的健康亲属中观察到免疫球蛋白异常的发生率增加。在大多数情况下,已知患者的家庭成员中骨髓瘤诊断的时间间隔在4年以内。这些观察结果,再加上社区中骨髓瘤聚集发生以及配偶中出现骨髓瘤的报告,增加了存在环境因素(病毒?)的可能性,这种因素可能在遗传易感性个体中促成骨髓瘤的发病机制。多发性骨髓瘤应被列入家族病史相关且遗传因素以及可能的环境因素可能在发病机制中起作用的肿瘤性疾病名单中。

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