Shibusawa Motoharu, Kidoguchi Keisuke, Tanimoto Tetsuya
IMS group Shinmatsudo Central General Hospital, Department of Hematology, Chiba, Japan
Saga-ken medical centre KOSEIKAN, Saga, Japan
Epstein-Barr virus (EBV)-positive diffuse large B cell lymphoma (DLBCL) is known for distinct clinical features. This disease was formerly designated as EBV-positive DLBCL of the elderly, but the restriction to elderly patients has been removed, and the World Health Organization classification revised 4th edition lists EBV-positive DLBCL, not otherwise specified (NOS). The frequency of EBV-positive DLBCL among DLCBL is about 2.5–14.0%, with higher incidence among East Asians. Most cases occur in patients aged over 50 years old with male predominance. The clinical characteristics of EBV-positive DLBCL, compared to those with EBV-negative DLBCL, include association with older age, more advanced clinical stage, a higher rate of extranodal involvement, and worse performance status. It is usually treated with R-CHOP, consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. EBV-positive DLBCL shows an inferior prognosis with R-CHOP as compared with EBV-negative DLBCL. Furthermore, high EBV-DNA load and positivity of EBV-encoded RNA in biopsy specimens are associated with a worse prognosis.
爱泼斯坦-巴尔病毒（EBV）阳性弥漫性大B细胞淋巴瘤（DLBCL）以其独特的临床特征而闻名。这种疾病以前被指定为老年EBV阳性DLBCL，但对老年患者的限制已被取消，世界卫生组织第4版修订分类列出了未另行指定（NOS）的EBV阳性DLBCL。EBV阳性DLBCL在弥漫性大B细胞淋巴瘤中的发生率约为2.5%-14.0%，在东亚人群中发病率更高。大多数病例发生在50岁以上的患者中，男性居多。与EBV阴性DLBCL相比，EBV阳性DLBCL的临床特征包括与年龄较大、临床分期更晚、结外受累率更高以及体能状态更差有关。它通常采用R-CHOP方案治疗，该方案由利妥昔单抗、环磷酰胺、多柔比星、长春新碱和泼尼松组成。与EBV阴性DLBCL相比，EBV阳性DLBCL采用R-CHOP方案治疗时预后较差。此外，活检标本中高EBV-DNA载量和EBV编码RNA阳性与预后较差有关。
