Abstract
The contribution of Epstein-Barr virus (EBV) to the development of specific types of benign lymphoproliferations and malignant lymphomas has been extensively studied since the discovery of the virus over the last 50 years. The importance and better understanding of the EBV-associated lymphoproliferative disorders (LPD) of B, T or natural killer (NK) cell type has resulted in the recognition of new entities like EBV+ mucocutaneous ulcer or the addition of chronic active EBV (CAEBV) infection in the revised 2016 World Health Organization (WHO) lymphoma classification. In this article, we review the definitions, morphology, pathogenesis, and evolving concepts of the various EBV-associated disorders including EBV+ diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS), EBV+ mucocutaneous ulcer, DLBCL associated with chronic inflammation, fibrin-associated DLBCL, lymphomatoid granulomatosis, the EBV+ T and NK-cell LPD of childhood, aggressive NK leukaemia, extranodal NK/T-cell lymphoma, nasal type, and the new provisional entity of primary EBV+ nodal T- or NK-cell lymphoma. The current knowledge regarding the pathogenesis of B-cell lymphomas that can be EBV-associated including Burkitt lymphoma, plasmablastic lymphoma and classic Hodgkin lymphoma will be also explored.
Highlights
Epstein-Barr virus (EBV) is a ubiquitous gamma herpes virus with tropism for B cells
The focus of this review is to describe the clinico-pathological features of the well-known B, T- and natural killer (NK)-cell EBV-associated lymphomas and lymphoproliferations in non-immunocompromised hosts [13]
The entities in which EBV is detectable but does not represent the disease defining feature including plasmablastic lymphoma (PBL), BL and classic Hodgkin lymphoma (CHL) will be addressed. Those lymphomas in immunosuppressed patients, where EBV is considered a non-essential component of lymphomagenesis (e.g., the spectrum of post-transplant lymphoproliferative disorders (PTLD) or those associated with primary immunodeficiencies) are beyond the scope of this review
Summary
Epstein-Barr virus (EBV) is a ubiquitous gamma herpes virus with tropism for B cells. EBV is the most common persistent virus infection in humans with approximately 95% of the world’s population showing an asymptomatic life-long carrier status [1] This sustained life-long latent infection is the result of the unique interaction of EBV with B cells, memory B cells, which are the EBV reservoir in healthy individuals [2,3]. Latency III involves the unrestricted expression of all nine latent genes including six EBV-encoded nuclear antigens (EBNA1, 2, 3A–C and LP) and three latent membrane proteins (LMP1, LMP2A and LMP2B). This latency program occurs only during acute EBV infection or in severely immunodeficient individuals.
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