Concordance for hodgkin's disease in identical twins suggesting genetic susceptibility to the young-adult form of the disease

Abstract

Background: 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. Methods: 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. Results: 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 disease was observed. Conclusions: Genetic susceptibility underlies Hodgkin's disease in young adulthood., Hodgkin's disease is likely to have different causes in young adulthood and old age.1 The characteristic clinical presentation and histopathological appearance suggest that in young adults the disease is initiated by an environmental exposure, possibly to an infectious agent. The age-specific incidence during young adulthood varies over time and according to place and social class, much like that of infections. Those affected have a small average number of siblings, may not have had the common childhood infections, and are likely to have a history of infectious mononucleosis.2 These findings have been interpreted to suggest that Hodgkin's disease may occur as… © 1995, Massachusetts Medical Society. All rights reserved.

Publication Title

New England Journal of Medicine

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