Hawkins M M, Draper G J, Winter D L
Childhood Cancer Research Group, University of Oxford, UK.
Br J Cancer. 1995 Jun;71(6):1335-9. doi: 10.1038/bjc.1995.259.
Understanding the extent to which childhood leukaemia and non-Hodgkin lymphomas are heritable is important to the survivors of these diseases, their families and clinicians who provide genetic counselling. Such understanding is also relevant to the possibility raised by Gardner et al. (1990, Br. Med. J., 300, 423-429) that paternal preconception irradiation may be an aetiological factor in these diseases. No malignant neoplasm was diagnosed among 382 offspring of survivors of childhood leukaemia and non-Hodgkin lymphoma followed up for a median period of 5.8 years, the largest available cohort of such offspring. These data indicate that it is unlikely that the risk of a malignant neoplasm occurring in the offspring exceeds eight times that expected in the general population. Similarly, the risk of leukaemia and non-Hodgkin lymphoma among offspring is unlikely to exceed 21 times that expected. The proportion of survivors of childhood leukaemia and non-Hodgkin lymphoma with the heritable form of these diseases is unlikely to exceed 5%, assuming an autosomal dominant pattern of transmission, with penetrance of at least 70% and that all heritable cases develop by age 15 years. The best (i.e. at present most likely) estimates of these risks are of course much lower. There was no evidence of an excess of congenital abnormalities among the offspring and the sex ratio was similar to that expected from the general population.
了解儿童白血病和非霍奇金淋巴瘤的遗传程度,对于这些疾病的幸存者、他们的家人以及提供遗传咨询的临床医生来说非常重要。这种了解也与加德纳等人(1990年,《英国医学杂志》,300卷,423 - 429页)提出的父亲受孕前接受辐射可能是这些疾病的病因之一这一可能性相关。在对382名儿童白血病和非霍奇金淋巴瘤幸存者的后代进行了为期5.8年的随访中,未诊断出任何恶性肿瘤,这是此类后代中规模最大的可用队列。这些数据表明,后代发生恶性肿瘤的风险不太可能超过一般人群预期风险的八倍。同样,后代患白血病和非霍奇金淋巴瘤的风险也不太可能超过预期风险的21倍。假设疾病以常染色体显性遗传模式传递,外显率至少为70%,且所有遗传性病例在15岁前发病,那么儿童白血病和非霍奇金淋巴瘤幸存者中患有这些疾病遗传形式的比例不太可能超过5%。当然,这些风险的最佳(即目前最有可能的)估计值要低得多得多。在这些后代中没有证据表明先天性异常增多,且性别比例与一般人群预期的相似。