EBV-Driven Lymphoproliferative Disorders and Lymphomas of the Gastrointestinal Tract: A Spectrum of Entities with a Common Denominator (Part 3)

Abstract

EBV is the first known oncogenic virus involved in the development of several tumors. The majority of the global population are infected with the virus early in life and the virus persists throughout life, in a latent stage, and usually within B lymphocytes. Despite the worldwide diffusion of EBV infection, EBV-associated diseases develop in only in a small subset of individuals often when conditions of immunosuppression disrupt the balance between the infection and host immune system. EBV-driven lymphoid proliferations are either of B-cell or T/NK-cell origin, and range from disorders with an indolent behavior to aggressive lymphomas. In this review, which is divided in three parts, we provide an update of EBV-associated lymphoid disorders developing in the gastrointestinal tract, often representing a challenging diagnostic and therapeutic issue. Our aim is to provide a practical diagnostic approach to clinicians and pathologists who face this complex spectrum of disorders in their daily practice. In this part of the review, the chronic active EBV infection of T-cell and NK-cell type, its systemic form; extranodal NK/T-cell lymphoma, nasal type and post-transplant lymphoproliferative disorders are discussed.

Keywords: Epstein–Barr virus; chronic active EBV infection; extranodal NK/T-cell lymphoma; nasal type; post-transplant lymphoproliferative disorders.

Figure 1

(a) PTLD: Medium power view showing a mucosal infiltrate with preserved tissue architecture (HE, 20× magnification); (b) PTLD: CD3 staining highlighting the mucosal infiltrate (immunohistochemical staining, 20×); (c) PTLD: EBER positivity (in situ hybridization; 40× magnification); (d) ENKTL, nasal-type: low power view showing the neoplastic infiltrate diffusely involving the intestinal wall; in the inset: details of neoplastic cells (HE, 10× magnification; inset, 40× magnification); (e) ENKTL, nasal-type: CD56 expression (immunohistochemical staining, 10× magnification); (f) ENKTL, nasal-type: EBER positivity (in situ hybridization; 10× magnification). All images are originals from Prof. S.A.P.