Abstract
Hodgkin lymphoma (HL) is a lymphatic malignancy with an estimated annual incidence of 80,000 cases world-wide. Modern therapy promises 5-year survival rates exceeding 90% for patients with early-stage disease. However, such treatment is not universally accessible, and each year HL causes around 25,000 deaths globally.
HL incidence displays considerable age-specific variation both within and between populations. In particular, a bimodal age distribution with separate incidence peaks in younger adults and in older adults, respectively, is characteristic of socio-economically affluent populations in the Western world.
Along with other epidemiological evidence, this has fostered hypotheses suggesting that etiologically heterogeneous HL variants exist and that HL in children and younger adults is of an infectious origin.
Evidence is growing that the presence and absence, respectively, of Epstein-Barr virus (EBV) in the malignant cells distinguish between etiologically distinct HL variants. The prevalence of EBV-positive HL varies with sex, age, ethnicity, socio-economic environment as well as medical history in ways suggesting that host immune control of the viral infection is critical to the lymphoma development.
HL displays familial accumulation, which is mirrored in identified genetic risk loci, some of which are shared between EBV-positive and EBV-negative HL, while others are specific to either of the two lymphoma phenotypes.
In contrast to EBV-positive HL, much less is known about risk factors for EBV-negative HL. Because EBV-negative HL constitutes the majority of cases in the young adult incidence peak typical of affluent populations, characteristics of an affluent childhood disease environment remain the most promising area to explore in the search for risk factors.
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Hjalgrim, H., Jarrett, R.F. (2020). Epidemiology of Hodgkin Lymphoma. In: Engert, A., Younes, A. (eds) Hodgkin Lymphoma. Hematologic Malignancies. Springer, Cham. https://doi.org/10.1007/978-3-030-32482-7_1
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