Division of Molecular Genetic Epidemiology, German Cancer Research Center, Heidelberg, Germany;
Division of Molecular Genetic Epidemiology, German Cancer Research Center, Heidelberg, Germany; Division of Preventive Oncology, National Center for Tumor Diseases (NCT), Heidelberg, Germany;
Blood. 2015 Oct 22;126(17):1990-5. doi: 10.1182/blood-2015-04-639781. Epub 2015 Aug 26.
We aimed to provide the familial risk of classical Hodgkin lymphoma (HL) by relationship, histology, age at diagnosis, and sex. A cohort of 57,475 first-degree relatives of 13,922 HL patients diagnosed between 1955 and 2009 in 5 European countries was observed for HL incidence. The overall lifetime cumulative risk (CR) of HL in first-degree relatives of a patient with HL was 0.6%, which represents a threefold (standardized incidence ratio [SIR], 3.3; 95% confidence interval [CI], 2.8-3.9) increased risk over the general population risk. The risk in siblings (6.0-fold; 95% CI, 4.8- to 7.4-fold) was significantly higher than in parents and/or children (2.1-fold; 95% CI, 1.6- to 2.6-fold). Very high lifetime risk of HL was found for those with multiple affected first-degree relatives (13-fold; 95% CI, 2.8- to 39-fold) and for same-sex twins (57-fold; 95% CI, 21- to 125-fold). We found high familial risks between some concordant histologic subtypes of HL such as lymphocyte-rich (81-fold; 95% CI, 30- to 177-fold) and nodular sclerosis (4.6-fold; 95% CI, 2.9- to 7.0-fold) and also between some discordant subtypes. The familial risk in sisters (9.4-fold; 95% CI, 5.9- to 14-fold) was higher than in brothers (4.5-fold; 95% CI, 2.9- to 6.7-fold) or unlike-sex siblings (5.9-fold; 95% CI, 4.3- to 8.1-fold). The lifetime risk of HL was higher when first-degree relatives were diagnosed at early ages (before age 30 years). This study provides tangible absolute risk estimates for relatives of HL patients, which can be used as a sex-, age-, and family history-based risk calculator for classical HL by oncologists and genetic counselors.
我们旨在通过亲属关系、组织学、诊断时的年龄和性别来提供经典霍奇金淋巴瘤 (HL) 的家族风险。在 5 个欧洲国家，对 1955 年至 2009 年间诊断出的 13922 例 HL 患者的 57475 名一级亲属进行了 HL 发病率的观察。HL 患者一级亲属的终生累积 HL 风险 (CR) 为 0.6%，与一般人群的风险相比，风险增加了三倍（标准化发病比 [SIR]，3.3；95%置信区间 [CI]，2.8-3.9）。同胞（6.0 倍；95%CI，4.8-7.4 倍）的风险明显高于父母和/或子女（2.1 倍；95%CI，1.6-2.6 倍）。对于那些有多个一级亲属患病的人（13 倍；95%CI，2.8-39 倍）和同性别双胞胎（57 倍；95%CI，21-125 倍），HL 的终生风险非常高。我们发现，HL 的一些一致组织学亚型之间存在较高的家族风险，例如富含淋巴细胞（81 倍；95%CI，30-177 倍）和结节性硬化症（4.6 倍；95%CI，2.9-7.0 倍），以及一些不一致的亚型之间。姐妹（9.4 倍；95%CI，5.9-14 倍）的家族风险高于兄弟（4.5 倍；95%CI，2.9-6.7 倍）或异性别兄弟姐妹（5.9 倍；95%CI，4.3-8.1 倍）。当一级亲属在年轻时（30 岁之前）被诊断出时，HL 的终生风险更高。这项研究为 HL 患者的亲属提供了切实的绝对风险估计，肿瘤学家和遗传咨询师可以使用这些估计值来制作基于性别、年龄和家族史的经典 HL 风险计算器。
