EBV-Driven Lymphoproliferative Disorders and Lymphomas of the Gastrointestinal Tract: A Spectrum of Entities with a Common Denominator (Part 2)

• Main Authors: Magda Zanelli, Francesca Sanguedolce, Andrea Palicelli, Maurizio Zizzo, Giovanni Martino, Cecilia Caprera, Valentina Fragliasso, Alessandra Soriano, Luca Valle, Stefano Ricci, Fabrizio Gozzi, Luca Cimino, Alberto Cavazza, Francesco Merli, Stefano A. Pileri, Stefano Ascani
• Format: Article
• Language: English
• Published: MDPI AG 2021-09-01
• Series: Cancers
• Subjects: Epstein–Barr virus; plasmablastic lymphoma; primary effusion lymphoma; Burkitt lymphoma
• Online Access: https://www.mdpi.com/2072-6694/13/18/4527

Epstein–Barr virus (EBV) is a common pathogen infecting people primarily early in life. The virus has the ability to persist throughout a person’s life, usually in B lymphocytes. Conditions of immunodeficiency as well as the introduction of immunosuppressive therapies and the advent of transplant technologies has brought immunodeficiency-associated lymphoproliferative disorders into view, which are often driven by EBV. The group of EBV-associated lymphoproliferative disorders includes different entities, with distinct biological features, ranging from indolent disorders, which may even spontaneously regress, to aggressive lymphomas requiring prompt and adequate treatment. These disorders are often diagnostically challenging due to their overlapping morphology and immunophenotype. Both nodal and extra-nodal sites, including the gastrointestinal tract, may be involved. This review, divided in three parts, summarizes the clinical, pathological, molecular features and treatment strategies of EBV-related lymphoproliferative disorders occurring in the gastrointestinal tract and critically analyzes the major issues in the differential diagnosis. In this part of the review, we discuss plasmablastic lymphoma, extra-cavitary primary effusion lymphoma and Burkitt lymphoma.