Nodal EBV+ cytotoxic T-cell lymphoma: A literature review based on the 2017 WHO classification

• Published in: Journal of Clinical and Experimental Hematopathology Vol. 60; no. 2; pp. 30 - 36
• Main Authors: Kato, Seiichi, Yamashita, Daisuke, Nakamura, Shigeo
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society for Lymphoreticular Tissue Research 01.01.2020; JSLRT
• Subjects: cytotoxic molecule; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - pathology; Epstein-Barr Virus Infections - therapy; Epstein-Barr Virus Infections - virology; Epstein–Barr virus; Herpesvirus 4, Human - isolation & purification; Humans; Immunophenotyping; Lymphoma, Extranodal NK-T-Cell - classification; Lymphoma, Extranodal NK-T-Cell - pathology; Lymphoma, Extranodal NK-T-Cell - therapy; Lymphoma, Extranodal NK-T-Cell - virology; Lymphoma, T-Cell, Peripheral - classification; Lymphoma, T-Cell, Peripheral - pathology; Lymphoma, T-Cell, Peripheral - therapy; Lymphoma, T-Cell, Peripheral - virology; nodal cytotoxic T-cell lymphoma; programmed cell-death ligand 1; Review; T-cell receptor phenotype; T-Lymphocytes, Cytotoxic - pathology; T-Lymphocytes, Cytotoxic - virology; World Health Organization; nodal cytotoxic T-cell lymphoma; T-cell receptor phenotype; cytotoxic molecule; Epstein–Barr virus; programmed cell-death ligand 1
• ISSN: 1346-4280; 1880-9952
• DOI: 10.3960/jslrt.20001

Nodal Epstein-Barr virus (EBV)-positive cytotoxic T-cell lymphoma (CTL) is a primary nodal peripheral T-cell lymphoma (PTCL) characterized by a cytotoxic phenotype and EBV on the tumor cells. This disease reportedly accounts for 21% of PTCL not otherwise specified (NOS). However, few nodal EBV+ lymphomas have been documented in detail. Nodal EBV+ CTL and nasal-type NK/T-cell lymphoma (NKTL) both exhibit cytotoxic molecule expression and EBV positivity on the tumor cells; however, nodal EBV+ CTL is characterized as a systemic disease without nasopharyngeal involvement, and exhibits a CD8+/CD56− phenotype distinct from NKTL. The clinicopathological uniqueness of nodal EBV+ CTL is further supported by its T-cell origin in most reported cases. In the 2008 WHO classification, it was unclear whether nodal EBV+ CTL should be classified as PTCL or NKTL. However, based on additional data, the 2017 revision classifies nodal EBV+ CTL as PTCL. In the present review, we focus on the clinicopathological characteristics of nodal EBV+ CTL, discuss the relationship between chronic active EBV infection and nodal EBV+ lymphoma, and highlight future perspectives regarding the treatment of this disease.