Beltran Brady E, Castro Denisse, Paredes Sally, Miranda Roberto N, Castillo Jorge J
Department of Oncology and Radiotherapy, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru.
Centro de Investigación de Medicina de Precision, Universidad San Martin de Porres, Lima, Peru.
Am J Hematol. 2020 Apr;95(4):435-445. doi: 10.1002/ajh.25760. Epub 2020 Feb 29.
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 (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.
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.
爱泼斯坦-巴尔病毒阳性（EBV+）弥漫性大B细胞淋巴瘤（DLBCL），未另行规定（NOS）是2016年世界卫生组织淋巴样肿瘤分类中包含的一种实体。EBV+ DLBCL，NOS，是一种与慢性EBV感染相关的侵袭性B细胞淋巴瘤，采用标准化疗方法预后较差。
通过仔细的病理评估进行诊断。检测EBV编码的RNA（EBER）被认为是诊断的标准方法；然而，尚未确定阳性的明确临界值。鉴别诊断包括浆母细胞淋巴瘤（PBL）、与慢性炎症相关的DLBCL和原发性渗出性淋巴瘤（PEL）等。
国际预后指数（IPI）和大山评分可用于风险分层。大山评分包括年龄>70岁和存在B症状。CD30和PD-1/PD-L1的表达正成为潜在的不良但可靶向的生物标志物。
EBV+ DLBCL，NOS患者应按照与EBV阴性DLBCL患者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的预后可能比EBV阴性DLBCL更差。在EBV+ DLBCL，NOS患者的治疗中，有机会研究和开发靶向治疗。
