What Can we Learn About the Aetiology of Hodgkin’s Disease from Family Studies?
Family studies have given us an important tool in understanding the aetiology of cancer. There are many examples, such as malignant melanoma and nasopharyngeal carcinoma, where the interaction of genetic and environmental factors is important in producing the observed pattern of disease. In the case of malignant melanoma, for example, a subset of the population has been identified with hereditary predisposition to melanoma associated with dysplastic nevi (Clark et al., 1978; Reimer et al., 1978). The pattern of inheritance appears to be dominant with approximately 90% penetrance. Environment plays a role in this genetically determined malignancy since the risk of melanoma in people with dysplastic nevi is related to the number of sunburns. Education and counselling of susceptible family members regarding sun exposure apparently changes their exposure patterns. Within several years of less sun exposure, the number of new melanomas and dysplastic nevi decrease in the individuals following skin care guidelines (Tucker, 1986). In nasopharyngeal carcinoma, the genetic aspects and the environmental aspects remain speculative. HLA loci have been linked to a putative disease-susceptibility gene in some specific multiple case families. The putative environmental factors include Epstein-Barr virus (EBV), exposure to nitrosamines, cigarette smoke, and other agents (Hildesheim and Levine, 1993). Nasopharyngeal carcinoma appears to be in the middle of a spectrum ranging from ataxia telangiectasia and xeroderma pigmentosum, where genetics appear to be the most important contributing factor, to adult T-cell leukaemia/lymphoma where the exposure to the oncogenic virus, HTLV-I, at an early age is clearly far more important than genetic susceptibility (Murphy et al., 1989).
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