Familial aggregation of Hodgkin lymphoma and related tumors
Familial aggregation of Hodgkin lymphoma and related tumors
复制标题
DOI：
10.1002/cncr.20189
复制
发表时间：
2004-05-01
期刊：
影响因子：
6.2
通讯作者：
Linet, MS
中科院分区：
文献类型：
--
作者：
Goldin, LR;Pfeiffer, RM;Linet, MS
背景资料。遗传因素在霍奇金淋巴瘤(HL)病因中的重要性已被家系和人群研究所证实。方法：采用边缘生存模型比较了5047名HL患者的15,799名一级亲属与10,078名对照先证者的32,117名一级亲属和2429名HL患者的7185名一级亲属与8,495名丹麦对照先证者的27,434名一级亲属的淋巴增殖性恶性肿瘤的诊断。结果：两个人群中HL患者亲属的HL风险显著增加，相对危险度为3.47(95%可信区间[95%CI]，瑞典为1.77-6.80)，丹麦为2.55(95%可信区间，1.01-6.45)，合并估计为3.11(95%可信区间，1.82-5.29)。在瑞典，患者亲属患慢性淋巴细胞白血病和非霍奇金淋巴瘤(男性)的风险也显著增加。男性的相对风险高于女性，患者的兄弟姐妹的相对风险高于患者的父母和子女。结论HL是一种重要的家族成员，其发病年龄在40岁以上的患者家族中、男性和兄弟姐妹中更为强烈，它与一些(但不是其他)淋巴增殖性恶性肿瘤有共同之处。然而，对于新发HL或受影响患者的一级亲属来说，累积的终身风险非常小。
BACKGROUND. The importance of genetic factors in the etiology of Hodgkin lymphoma (HL) has been suggested by family and population studies. However, the spectrum of malignancies associated with common genetic etiology and the effects of gender and age on familial risk have not been established.METHODS. Diagnoses of lymphoproliferative malignancies were compared in 15,799 first-degree relatives of 5047 patients with HL versus 32,117 first-degree relatives of 10,078 control probands from Sweden and in 7185 first-degree relatives of 2429 patients with HL versus 27,434 first-degree relatives of 8,495 control probands from Denmark using marginal survival models.RESULTS. The risk of HL in relatives of patients with HL was increased significantly in both populations, with relative risks of 3.47 (95% confidence interval [95% CI], 1.77-6.80) in Sweden and 2.55 (95% CI, 1.01-6.45) in Denmark and a pooled estimate of 3.11 (95%CI, 1.82-5.29). In Sweden, risks for relatives of patients also were increased significantly for chronic lymphocytic leukemia and non-Hodgkin lymphoma (in males). Relative risks were higher in males compared with females and in siblings of patients compared with parents and offspring of patients. Relatives of patients with earlier-onset disease were at higher risk for HL.CONCLUSIONS. HL has an important familial component, which is stronger in families of affected individuals age < 40 years, in males, and in siblings, and it is shared with some (but not other) lymphoproliferative malignancies. The cumulative lifetime risks are very small, however, for the development of HL de novo or in first-degree relatives of affected patients.