Multicentric primary intestinal EBV-positive diffuse large B cell lymphoma of the elderly presenting with perforation

Epstein-Barr virus (EBV)-positive diffuse large B cell lymphoma (DLBCL) of the elderly is a recently defined subgroup of DLBCL in the 2008 WHO classification of lymphoid neoplasms. It is characterized by an EBV-positive clonal B cell lymphoproliferation that occurs in patients aged over 50 years without any known immunodeficiency. This disease shows frequent extranodal involvement, a morphological spectrum from polymorphous to monomorphic large cells, and a poor prognosis. We reported the case of a 75-year-old male who had a primary intestinal multicentric EBV(+) DLBCL and presented with perforation, the first multicentric case in the literature. The monomorphic tumor cells exhibited extensive necrosis and expressed CD20, CD138, and EBNA2 with diffuse positivity for EBV by in situ hybridization. Quantitative real-time PCR revealed a low serum EBV viral load. The patient passed away in 10 days. His poor outcome was probably due to perforation, age over 70, the presence of B symptoms, poor general condition, and high performance score.