Epstein-barr virus-positive diffuse large B-cell lymphoma of the elderly: what we know so far

Abstract

Epstein-Barr virus-positive (EBV-positive) diffuse large B-cell lymphoma (DLBCL) of the elderly is a newly described lymphoproliferative disorder recently included as a "provisional" entity in the most current WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. The objective of this review is to provide a thorough and current summary of the existing knowledge of this subtype of DLBCL. We will review and discuss the incidence of EBV expression in DLBCL, the pathogenesis behind EBV-driven malignant transformation of B cells, the different EBV latency patterns associated with DLBCL, the distinct pathologic characteristics of EBV-positive DLBCL, the potential predictive and prognostic value of EBV tumoral status in patients with DLBCL, and potential strategies for the treatment of this rare entity, which is characterized by a suboptimal response to therapy and poor survival rate.

Conflict of interest statement

Disclosures: Jorge J. Castillo: None; Brady E. Beltran: None; Roberto N. Miranda: None; Semra Paydas: None; Eric S. Winer: None; James N. Butera: None

The content of this article has been reviewed by independent peer reviewers to ensure that it is balanced, objective, and free from commercial bias. No financial relationships relevant to the content of this article have been disclosed by the authors or independent peer reviewers.

Figures

Figure 1.

Pathophysiology of EBV-positive DLBCL of the elderly. Abbreviations: DLBCL, diffuse large B-cell lymphoma; EBV, Epstein-Barr virus.

Figure 2.

Pathologic characteristics of EBV-positive DLBCL of the elderly. (A): EBV-positive DLBCL of the elderly infiltrating soft tissue. Hematoxylin and eosin, ×100. (B): Monomorphic pattern displaying large cells with immunoblastic features, characterized by central prominent nucleoli. Hematoxylin and eosin, ×400. (C): Polymorphic pattern displaying large cells admixed with abundant reactive inflammatory cells, mainly small lymphocytes. Hematoxylin and eosin, ×400. (D): CD20. Positivity for CD20 is consistent with a B-cell lineage. Immunohistochemistry, ×400. (E): MUM-1 (nuclear pattern). Most EBV-positive DLBCL of the elderly are positive for MUM-1, consistent with a nongerminal center cell phenotype. Immunohistochemistry, ×400. (F): EBV-encoded RNA (EBER) (nuclear pattern). In situ hybridization, ×400. Abbreviations: DLBCL, diffuse large B-cell lymphoma; EBV, Epstein-Barr virus.