The rarity of familial Hodgkin lymphoma (HL) has hampered detailed analyses of familial clustering. We aimed to provide the familial risk of 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 five European countries was followed for HL incidence. Standardized incidence ratios (SIRs) were calculated using histology-, age-, sex-, period-, and country-specific incidence rates as the reference. The lifetime cumulative risks (CR) were also calculated. The overall CR of HL in first-degree relatives of a patient with HL was 0.6%, which represents a 3-fold (SIR=3.3, 95%CI=2.8–3.9) increased risk over the general population risk. The risk in siblings (6.0-fold; 4.8–7.4) was significantly higher than in parents/children (2.1-fold; 1.6–2.6). Very high lifetime risk of HL was found for those with multiple affected first-degree relatives (13-fold; 2.8-39) and for same-sex twins (57-fold; 21-125). We found high familial risks between some concordant histological subtypes of HL [lymphocyte-rich (81-fold, 30–177) and nodular sclerosis (4.6-fold, 2.9–7.0)] and also between some discordant subtypes. The familial risk in sisters (9.4-fold; 5.9–14) was higher than in brothers (4.5-fold; 2.9–6.7) or unlike-sex siblings (5.9-fold; 4.3–8.1). The lifetime risk of HL was higher when first-degree relatives were diagnosed at early ages (before age 30). 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 Hodgkin lymphoma by oncologists and genetic counselors. 淋巴瘤会遗传吗?到目前为止,这个问题还没有明确的答案。最近,来自欧洲的一项研究部分回答了关于霍奇金淋巴瘤家族遗传的问题。这项研究追踪了北欧五个国家13922位霍奇金患者以及57475位直系亲属的情况,确诊的时间跨度超过了半个世纪。总的来说,结论是:直系亲属的终生累计风险为0.6%,是一般人群的3倍,其中,兄弟姐妹的风险达到6倍,而子女的风险为2.1倍。如果直系亲属有多位患病的话,风险会高达13倍。从组织学分类来说,富于淋巴细胞型的风险最高,达到81倍,结节硬化性4.6倍。如果直系亲属患病年龄低于30岁的话,风险也会相对高一些。 解读:由于人种不同,欧洲的结果恐怕不能直接在中国套用;亲属风险增加的原因应该不能单纯用遗传来解释,因为霍奇金与病毒感染例如EB病毒感染有一定关系;即便终生累计风险升高,其实发病率依然是很低的,特别是考虑到亚洲人种的霍奇金发病率比西方白种人要低。 我找不到很可靠的统计数据,只能根据一些有出处,还算靠谱的数据来推算。根据北京市卫生局公布的统计数字,2010年北京市淋巴瘤发病率是9.13/10万人,而1983年中国全国淋巴瘤协作组统计的霍奇金在全部淋巴瘤的比例是8%,假设这些年来这个比例并没有太大的变化,那么中国一般人群每年得上霍奇金的概率大约是0.000007304,假设平均预期寿命是80岁,那么终身风险是0.00058432,在这个基础上,风险增加几倍,似乎也没什么大不了的。
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