In Europe expression of EBNA2 is associated with poor survival in EBV-positive diffuse large B-cell lymphoma of the elderly

Abstract

Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL) of the elderly occurs by definition in patients above the age of 50 years without any known underlying immunodeficiency. We investigated the incidence and clinical relevance of this subtype in Europe with special attention to the EBV-latency type. Among the 598 DLBCL, 15 EBV-positive lymphomas fulfilling the criteria of EBV-positive DLBCL of the elderly were identified (2.5%). Patients with EBV-positive DLBCL expressing EBNA2 showed a significantly poorer overall survival than patients with EBNA2-negative EBV-positive DLBCL (p = 0.0156). The incidence of EBV-positive DLBCL of the elderly in Europe is much lower than in Asian countries (2.5% of all cases of DLBCL). Interestingly, the likelihood of EBV positivity did not increase with age in patient above 50 years. Among EBV-positive DLBCL of the elderly a subgroup with EBV-latency type III expressing EBNA2 can be identified, which shows a poor outcome.

Keywords::

• Age
• DLBCL
• Ebstein-Barr virus
• latency type

Acknowledgements

The authors would like to thank Charlotte Botz-von Drathen and Olivera Batic for their excellent technical assistance. The work was supported by the Dr Werner Jackstädt-Stiftung Junior Excellence Research Group “Mechanisms of B-cell lymphoma development at old age as a basis for age adjusted therapeutic strategies”. We are indebted to the physicians who treated the patients and the pathologists who referred the cases to us for contributing specimens and clinical information and to Kay Dege for editing the manuscript. CSL, AB, LQM, SH, AT, IO and WK selected cases and analyzed slides. MK performed statistical analysis. RT provided clinical data. CSL and WK wrote the manuscript. All authors have approved the final version of the manuscript. The authors have no conflict of interest to report.

Potential conflict of interest

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Supplementary material available online

Supplementary Tables I–III.