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Hodgkin’s disease was recently designated Hodgkin lymphoma (HL) in the World Health Organization Classification.1 The National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) population based registries estimate that 7900 new cases are diagnosed annually in the USA.2 Clues to its aetiology have been suggested by the bimodal age distribution; higher risks in males, in people with higher socioeconomic status, and in smaller families; and occurrence of Epstein-Barr virus in HL tumour cells.3 The importance of genetic factors is indicated by reports of multiply affected families from case series,4–6 a twin study,7 a case–control study,8 and population registry studies carried out in Utah,9 Denmark,10 Israel,11 and Sweden.12–14 We recently analysed data from registries in Sweden and Denmark and found significant familial aggregation of HL and other lymphoproliferative tumours.15 The relative risk for HL among first degree relatives of cases compared with controls was 3.1. Relative risks were higher in males compared with females, and in siblings of cases compared with parents and offspring. Relatives of earlier onset cases were at higher risk for HL and for all lymphoproliferative tumours and were also at higher risk for developing early onset tumours themselves. These findings are consistent with those seen from earlier case series studies but have the advantage of being from large, population based samples.
It is not known whether or how extrinsic risk factors interact with genetic susceptibility. Identifying inherited susceptibility genes is an important step towards defining the pathway(s) leading to development of HL and understanding its complex aetiology. There have been many studies of somatic mutations in HL tumour cells, but although there are associations with HLA types, specific germline genes causing susceptibility have not yet been identified. Early studies of HLA Class I alleles …
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Competing interests: none declared
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