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EBV‐positive diffuse large B‐cell lymphoma, not otherwise specified: 2020 update on diagnosis, risk‐stratification and management

2020; Wiley; Volume: 95; Issue: 4 Linguagem: Inglês

10.1002/ajh.25760

1096-8652

Brady Beltrán, Denisse Castro, Sally Rose Paredes, Roberto N. Miranda, Jorge J. Castillo,

CNS Lymphoma Diagnosis and Treatment

Abstract Disease Overview Epstein Barr virus‐positive (EBV+) diffuse large B‐cell lymphoma (DLBCL), not otherwise specified (NOS) is an entity included in the 2016 WHO classification of lymphoid neoplasms. EBV+ DLBCL, NOS, is an aggressive B‐cell lymphoma associated with chronic EBV infection, and a poor prognosis with standard chemotherapeutic approaches. Diagnosis The diagnosis is made through a careful pathological evaluation. Detection of EBV‐encoded RNA (EBER) is considered standard for diagnosis; however, a clear cutoff for positivity has not been defined. The differential diagnosis includes plasmablastic lymphoma (PBL), DLBCL associated with chronic inflammation and primary effusion lymphoma (PEL), among others. Risk‐Stratification The International Prognostic Index (IPI) and the Oyama score can be used for risk‐stratification. The Oyama score includes age >70 years and presence of B symptoms. The expression of CD30 and PD‐1/PD‐L1 are emerging as potential adverse but targetable biomarkers. Management Patients with EBV+ DLBCL, NOS, should be staged and managed following similar guidelines than patients with EBV‐negative DLBCL. EBV+ DLBCL, NOS, however, might have a worse prognosis than EBV‐negative DLBCL in the era of chemoimmunotherapy. There is an opportunity to study and develop targeted therapy in the management of patients with EBV+ DLBCL, NOS.