Age-related Epstein-Barr virus-associated B-cell lymphoproliferative disorders: Special references to lymphomas surrounding this newly recognized clinicopathologic disease

Epstein-Barr virus (EBV) is associated with some disease entities of malignant lymphomas, including Burkitt lymphoma, Hodgkin lymphoma, immunodeficiency-associated lymphoproliferative disorders (LPD), and a part of diffuse large B-cell lymphoma. We have recently identified a series of elderly patients with EBV-associated (or EBV+) B-cell LPD (B-LPD) showing similarities in many respects to immunodeficiency-associated LPD, although no evidence of underlying immunodeficiency was found. Therefore, the nosological category of senile or age-related EBV+ B-LPD has been proposed for those patients. A larger series of patients with this disease revealed that the relative ratios of such EBV+ B-LPD to all diffuse large B-cell lymphoma cases were higher with increasing with age, reaching a peak (20-30%) at >= 90 years of age, with a median of 71 years, providing additional evidence for our assertion that this disease may be related to immunological deterioration as a result of the aging process. This new disease entity is characterized pathologically by centroblasts, immunoblasts, and Hodgkin and Reed-Sternberg-like giant cells with a varying degree of reactive components, often posing therapeutic and diagnostic problems for hematologists and pathologists, respectively. The aim of the present review is to briefly summarize the overall clinicopathological profile of this newly recognized age-related (also called 'senile') EBV+ B-LPD and EBV+ Hodgkin lymphoma for a practical diagnostic approach.