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目的老年人EB病毒阳性弥漫大B细胞淋巴瘤(Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly,EBV+DLBCL-E)是2008版WHO淋巴造血组织肿瘤分类中新增加的暂定肿瘤类型,对其研究较少。本研究探讨EBV+DLBCL-E的临床病理特点、治疗和预后。方法选择2009-01-01-2013-12-30南京军区南京总医院确诊的4例EBV+DLBCL-E。活检淋巴结行常规病理形态学、免疫组织化学染色和EBV编码小核糖核酸(EBV-encoded small RNAs,EBER)原位杂交检查,根据2008版WHO标准确诊EBV+DLBCL-E。血浆EBV-DNA定量采用实时荧光定量PCR方法。患者接受联合化疗或免疫化疗。回顾性分析EBV+DLBCL-E的临床特点、病理形态、免疫组化染色、EBV-DNA水平以及治疗和预后。结果4例EBV+DLBCL-E患者,男女各2例。发病年龄53~69岁,中位年龄59.5岁。均有发热和体质量减轻等B症状,均以淋巴结病变合并结外病变起病,包括脾大,肺和鼻咽部肿块及胸腹腔积液。临床分期均为晚期,其中ⅢB期3例,ⅣB期1例。根据国际预后指数判定,高中危2例,高危2例。免疫组化示,肿瘤细胞表达CD20、CD79a和Pax-5等全B细胞标记,原位杂交检测肿瘤细胞EBER均阳性。Hans分型均为非生发中心型。肿瘤细胞增殖指数高,4例中3例的Ki-67达80%。血浆EBV-DNA检测均阴性。采用联合化疗后,仅1例部分缓解,余3例均疾病进展,诊断后生存时间仅3~10个月。结论 EBV+DLBCL-E作为一种独特亚型,具有多种提示预后不良的临床和免疫组化特点,侵袭性强,对联合化疗反应差,长期生存率低,急需研究以EB病毒为靶点的新治疗方法。
The purpose of the EBV-positive diffuse large B-cell lymphoma (Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly, EBV DLBCL-E) is a new tentative tentatively added to the 2008 WHO classification of lymphoid hematopoietic tumors Tumor types, less research on it. This study was to investigate the clinicopathological features, treatment and prognosis of EBV + DLBCL-E. Methods: Four cases of EBV + DLBCL-E confirmed by Nanjing General Hospital of Nanjing Military Command from January 2009 to January 2013 were selected. The biopsy lymph nodes were examined by routine histopathology, immunohistochemical staining and EBV-encoded small RNAs (EBER) in situ hybridization, and EBV + DLBCL-E was confirmed according to the 2008 WHO standard. Plasma EBV-DNA quantification using real-time fluorescence quantitative PCR method. Patients underwent combined chemotherapy or immunotherapy. The clinical features, histopathology, immunohistochemical staining, EBV-DNA levels and treatment and prognosis of EBV + DLBCL-E were retrospectively analyzed. Results 4 cases of EBV + DLBCL-E patients, male and female in 2 cases. Age 53 to 69 years old, the median age of 59.5 years old. Have fever and weight loss and other symptoms of B, all with lymph node disease combined with extranodal disease onset, including splenomegaly, lung and nasopharyngeal mass and pleural effusion. Clinical stage are advanced, of which Ⅲ B in 3 cases, Ⅳ B in 1 case. According to the international prognostic index, 2 were in high school and 2 were in high risk. Immunohistochemistry showed that the tumor cells expressed all B cell markers such as CD20, CD79a and Pax-5, and the EBER of the tumor cells was positive by in situ hybridization. Hans typing are non-germinal center type. The tumor cell proliferation index was high, and in 3 of 4 cases Ki-67 was 80%. Plasma EBV-DNA tests were negative. After combined chemotherapy, only 1 case of partial response, more than 3 cases of disease progression, survival time after diagnosis of only 3 to 10 months. Conclusion As a unique subtype, EBV + DLBCL-E has many clinical and immunohistochemical features suggesting poor prognosis, strong invasiveness, poor response to combined chemotherapy and long-term survival, and is urgently needed to target Epstein-Barr virus The new treatment.