Abstract
A proportion of cases of classical Hodgkin lymphoma (cHL) are associated with the Epstein–Barr virus (EBV). In these cases, EBV is present in all the Hodgkin and Reed–Sternberg (HRS) cells, the infection is clonal, and EBV proteins and noncoding RNAs are expressed. The known biological functions of two of these EBV proteins, LMP1 and LMP2A, suggest that they are likely to play a key role in the survival and reprogramming of HRS cells. EBV-associated and nonassociated cHL cases have distinct demographic features and risk factors for disease development; differences in the molecular pathogenesis of the two groups of cases are also emerging. EBV-associated cases are more common in early childhood and older adult age groups and account for only a quarter or less of the cases in the young adult incidence peak, which is characteristic of industrialized countries. Epidemiological evidence suggests that delayed exposure to a common childhood infectious agent may be involved in the etiology of young adult cHL. Herpesviruses and polyomaviruses are candidate agents but there is currently no evidence that members of the these virus families, with the exception of EBV, are directly involved. Modern molecular methods of virus discovery should help to determine whether a hitherto unknown virus is involved in EBV-negative cHL; this is important since identification of such an agent would open up possibilities for disease prevention as well as novel therapeutic targets.
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Abbreviations
- BART:
-
BamHI A rightward transcripts
- cHL:
-
Classical Hodgkin lymphoma
- EBER:
-
EBV-encoded small RNAs
- EBNA:
-
EBV nuclear antigen
- EBV:
-
Epstein–Barr virus
- HHV:
-
Human herpesvirus
- HL:
-
Hodgkin lymphoma
- HLA:
-
Human leukocyte antigen
- HRS:
-
Hodgkin and Reed–Sternberg
- LMP:
-
Latent membrane protein
- MCV:
-
Merkel cell polyomavirus
- MV:
-
Measles virus
- SNP:
-
Single nucleotide polymorphism
- TTV:
-
Torque teno virus
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Acknowledgments
To Scamp, my faithful old feline friend who died during the preparation of this manuscript. Thanks to Tina Rich for reading the manuscript. Work in our laboratory is supported by the Leukaemia Lymphoma Research and the Kay Kendall Leukaemia Fund.
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Jarrett, R.F. (2011). The Role of Viruses in the Genesis of Hodgkin Lymphoma. In: Engert, A., Horning, S. (eds) Hodgkin Lymphoma. Hematologic Malignancies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-12780-9_2
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