Abstract
Simple SummaryEpstein-Barr virus (EBV) contributes to the reactive and neoplastic lymphoid proliferation of B-, T-, and NK-cell lineages, which represent a vast clinicopathological spectrum ranging from indolent, self-limited disease to aggressive lymphomas. EBV-positive B-cell lymphoproliferative disorder (EBV+ B-LPD) is the most common of these diseases, accounting for 3% to 15% of diffuse large B-cell lymphomas. The spectrum of EBV+ B-LPD is expanding with advances in understanding immunosenescence and iatrogenic immunodeficiency in the era of immune-oncology. We review EBV+ B-LPD affecting the gastrointestinal tract with a focus on the PD-L1 expression in tumor and non-malignant immune cells to better understand this peculiar disease.Epstein-Barr virus positive B-cell lymphoproliferative disorder (EBV+ B-LPD) encompasses a broad clinicopathological spectrum and distinct clinical behavior that relatively favors the gastrointestinal (GI) tract. In this review, we provide an update on the clinicopathological features and biological behavior of EBV-positive mucocutaneous ulcer (EBVMCU) and primary EBV+ diffuse large B-cell lymphoma (DLBCL) of the GI tract. EBVMCU is a newly recognized entity but well known as an indolent and self-limited EBV+ B-LPD occurring in various immunodeficiencies. In contrast, EBV+ DLBCL constitutes the largest group of EBV+ B-LPDs and is regarded as an aggressive neoplasm. These two distinct diseases have historically been distinguished in the reappraisal of age-related EBV-associated B-LPDs but are challenging in routine practice regarding their differential diagnostic and therapeutic approaches. An increasing number of reports indicate that they are epidemiologically prevalent beyond western and eastern countries, but their comprehensive analysis is still limited. We also describe the PD-L1 positivity of tumorous large cells and non-malignant immune cells, which is relevant for the prognostic delineation among patients with primary DLBCL of the GI tract with and without EBV on tumor cells.
Highlights
Epstein-Barr virus (EBV) induces B-cell transformation, and disruption of a finely balanced relationship between the virus and host immune system can lead to EBV+ B-cell lymphoproliferative disorders (B-LPDs), which represent a wide and expanding clinicopathological spectrum ranging from indolent and self-limited disease to aggressive lymphoma
We further revealed that programmed cell death ligand 1 (PD-L1) expression on tumor cells and non-malignant immune cells has an opposite prognostic impact in patients with giDLBCL [12,35]
The focus of this review is to describe the clinicopathological features of EBV-associated lymphoproliferative disorders (LPDs), especially EBV-positive mucocutaneous ulcer (EBVMCU) and EBV+ diffuse large B-cell lymphoma (DLBCL), in light of the GI tract involvement and PD-L1 expression
Summary
Dojcinov et al recently divided EBV+ B-LPDs into five categories: infectious mononucleosis (IM); EBV+ DLBCL, not otherwise specified (NOS); EBVMCU; DLBCL-associated chronic inflammation (DLBCL-CI); lymphomatoid granulomatosis (LyG) [1]. The 2017 WHO classification of malignant lymphoma encompassed these diverse diseases and emphasized that EBV+ DLBCL, NOS often affects both young and elderly immunocompetent patients. EBV+ DLBCL often presents an aggressive clinical course with frequent extranodal disease. DLBCL-CI is a rare EBV-associated entity described in association with pyothorax with a history of underlying chronic inflammation and pursues an aggressive clinical course [25]. The focus of this review is to describe the clinicopathological features of EBV-associated lymphoproliferative disorders (LPDs), especially EBVMCU and EBV+ DLBCL, in light of the GI tract involvement and PD-L1 expression
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