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

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.