Mack T M, Cozen W, Shibata D K, Weiss L M, Nathwani B N, Hernandez A M, Taylor C R, Hamilton A S, Deapen D M, Rappaport E B
Department of Preventive Medicine, University of Southern California School of Medicine, Los Angeles.
N Engl J Med. 1995 Feb 16;332(7):413-8. doi: 10.1056/NEJM199502163320701.
Relatives of young adults with Hodgkin's disease are at increased risk of Hodgkin's disease, and lines of evidence implicate both inheritance and environment.
We have identified and followed 432 sets of twins affected by Hodgkin's disease. The number of cases of Hodgkin's disease observed before the age of 50 years in the healthy monozygotic and dizygotic twins of the patients with Hodgkin's disease was compared with the number expected from national age-specific incidence rates.
None of the 187 pairs of dizygotic twins became concordant for Hodgkin's disease, whereas 10 of the 179 pairs of monozygotic twins did; in 5 of these pairs, the second case appeared after the original ascertainment. During the observation period, 0.1 (monozygotic) and 0.1 (dizygotic) cases in the unaffected twins were expected. Monozygotic twins of patients with Hodgkin's disease thus had a greatly increased risk (standardized incidence ratio, 99; 95 percent confidence interval, 48 to 182), whereas no increase in the risk for dizygotic twins of patients with Hodgkin's was observed.
Genetic susceptibility underlies Hodgkin's disease in young adulthood.
霍奇金淋巴瘤青年患者的亲属患霍奇金淋巴瘤的风险增加,有证据表明遗传因素和环境因素均与之相关。
我们识别并追踪了432对患霍奇金淋巴瘤的双胞胎。将霍奇金淋巴瘤患者的健康同卵双胞胎和异卵双胞胎在50岁之前观察到的霍奇金淋巴瘤病例数与根据全国年龄特异性发病率预期的病例数进行比较。
187对异卵双胞胎中无一例患霍奇金淋巴瘤的情况一致,而179对同卵双胞胎中有10对一致;其中5对中,第二例病例在最初确诊后出现。在观察期内,预计未受影响的双胞胎中同卵双胞胎有0.1例、异卵双胞胎有0.1例患霍奇金淋巴瘤。因此,霍奇金淋巴瘤患者的同卵双胞胎患霍奇金淋巴瘤的风险大幅增加(标准化发病率为99;95%置信区间为48至182),而异卵双胞胎患霍奇金淋巴瘤的风险未增加。
遗传易感性是青年期霍奇金淋巴瘤的基础。