Division of Hematologic Malignancies, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts.
Department of Oncology and Radiotherapy, Hospital Nacional Edgardo Rebagliati Martins, and Research Center for Precision Medicine, Universidad San Martin de Porres Medical School, Lima, Peru.
Am J Hematol. 2018 Jul;93(7):953-962. doi: 10.1002/ajh.25112.
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.
The diagnosis is made through a careful pathological evaluation. Detection of EBV-encoded RNA is considered standard for diagnosis; however, a clear cutoff for positivity has not been defined. The differential diagnosis includes plasmablastic lymphoma, DLBCL associated with chronic inflammation, primary effusion lymphoma, HHV8+ DLBCL, NOS, and EBV+ mucocutaneuos ulcer.
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 is emerging as a potential adverse, and targetable, prognostic factor.
Patients with EBV+ DLBCL, NOS, should be staged and managed following similar guidelines than patients with EBV-negative DLBCL. EBV+ DLBCL, NOS, however, has 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.
EBV 阳性（EBV+）弥漫性大 B 细胞淋巴瘤（DLBCL），非特指型（NOS）是 2016 年 WHO 淋巴肿瘤分类中包含的一个实体。EBV+ DLBCL，NOS 是一种侵袭性 B 细胞淋巴瘤，与慢性 EBV 感染相关，且采用标准化疗方案预后较差。
通过仔细的病理评估做出诊断。检测 EBV 编码 RNA 被认为是诊断的标准；然而，尚未确定明确的阳性界限。鉴别诊断包括浆母细胞淋巴瘤、与慢性炎症相关的 DLBCL、原发性渗出性淋巴瘤、HHV8+ DLBCL，NOS 和 EBV+黏膜皮肤溃疡。
国际预后指数（IPI）和 Oyama 评分可用于风险分层。Oyama 评分包括年龄>70 岁和存在 B 症状。CD30 的表达正在成为一个潜在的不良预后因素，也是一个有治疗潜力的预后因素。
EBV+ DLBCL，NOS 患者应按照与 EBV 阴性 DLBCL 相似的指南进行分期和治疗。然而，在化疗免疫治疗时代，EBV+ DLBCL，NOS 的预后比 EBV 阴性 DLBCL 差。在 EBV+ DLBCL，NOS 的治疗中，有机会进行靶向治疗的研究和开发。
