EB 病毒阳性 (EBV) 弥漫性大 B 细胞淋巴瘤 (DLBCL)，未另有说明 (NOS) 是一种与 EBV 感染相关的侵袭性 B 细胞淋巴瘤，自 2016 年起被纳入 WHO 淋巴肿瘤分类。预后不良，在化学免疫治疗时代，结果似乎有所改善。通过细致的病理评估来确定诊断。检测 EBV 编码的 RNA (EBER) 是标准诊断方法。 ICC 2022 将 EBV DLBCL、NOS 指定为当 >80% 的恶性细胞表达 EBER 时发生，而 WHO-HAEM5 则强调大多数肿瘤细胞应为 EBER 阳性，但没有设定明确的阈值。鉴别诊断包括浆母细胞淋巴瘤、慢性炎症相关的DLBCL、原发性渗出性淋巴瘤等。国际预后指数（IPI）和Oyama评分可用于危险分层。 Oyama 评分包括年龄 >70 岁和是否存在 B 症状。 CD30 和 PD-1/PD-L1 的表达正在成为潜在不利但可靶向的生物标志物。 EBV DLBCL、NOS 患者应遵循与 EBV 阴性 DLBCL 患者类似的指南进行分期和管理。然而，在化学免疫治疗时代，EBV DLBCL，NOS 的预后可能比 EBV 阴性 DLBCL 更差。因此，建议在可能的情况下将患者纳入临床试验。有机会研究和开发 EBV DLBCL 患者管理的靶向治疗，NOS。© 2024 Wiley periodicals LLC。

Epstein Barr virus-positive (EBV+) diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS) is an aggressive B-cell lymphoma associated with EBV infection included in the WHO classification of lymphoid neoplasms since 2016. Although historically associated to poor prognosis, outcomes seem to have improved in the era of chemoimmunotherapy.The diagnosis is established through meticulous pathological evaluation. Detection of EBV-encoded RNA (EBER) is the standard diagnostic method. The ICC 2022 specifies EBV+ DLBCL, NOS as occurring when >80% of malignant cells express EBER, whereas the WHO-HAEM5 emphasizes that the majority of tumor cells should be EBER positive without setting a defined threshold. The differential diagnosis includes plasmablastic lymphoma, DLBCL associated with chronic inflammation, primary effusion lymphoma, among others.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.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. Therefore, inclusion of patients in clinical trials when available is recommended. There is an opportunity to study and develop targeted therapy in the management of patients with EBV+ DLBCL, NOS.© 2024 Wiley Periodicals LLC.