中国西南地区老年人EB病毒阳性弥漫大B细胞淋巴瘤的临床病理特点及预后分析

目的探讨中国西南地区老年人EB病毒阳性弥漫大B细胞淋巴瘤（DLBCL）的临床病理特点及预后相关因素。方法结合组织芯片技术，对42例老年人EB病毒阳性的DLBCL进行回顾性研究，包括临床特点总结、病理形态学复习、免疫组化染色以及预后相关因素分析。结果从586例DLBCL中筛选出42例（7.17%）EB病毒阳性DLBCL。患者发病年龄51 ~87岁，中位年龄62.5岁，男女之比为2.23∶1。其中69.05%（29/42）为淋巴结病变；30.95%（13/42）为结外病变，包括脾脏、胃、扁桃体、鼻腔和鼻咽部。其主要临床表现为淋巴结肿大、脾大、肝大、发热、乏力、体质量减轻。组织学亚型中绝大多数（90.48%，38/42）为多形性亚型，少数（9.52%，4/42）为单形性亚型，“星空”现象、血管浸润和肿瘤性坏死分别占21.43%（9/42）、47.62%（20/42）和45.24%（19/42）。其免疫表型分析示CD30、CD5、BCL-2、 P53、NF-κB/P65的阳性率分别为52.38%（22/42）、54.76%（23/42）、54.76%（23/42）、87.5%（35/40）和0%（0/40）；35例（83.33%）为Hans分型的non-GCB型。获得随访的23名患者中有14例（60.87%）患者死亡，中位生存时间为40个月，总体5年生存率为16.5%。多因素分析显示是否LDH升高、具有“星空”现象、肿瘤性坏死、BCL-2阳性表达与预后有关。结论中国西南地区老年人EB病毒阳性DLBCL的发生率不低，常见于淋巴结，以多形性组织学亚型多见，LDH升高、具有“星空”现象和BCL-2阳性者预后更差。

英文摘要:

Objective To investigate the clinicopathologic features, immunophenotype, and the prognosis related factors of Epstein-Barr virus (EBV) positive diffuse large B-cell lymphoma (DLBCL) in west-southern China. Methods There were 42 cases of EBV⁺ DLBCL in a total 586 DLBCL, the clinical and pathologic profiles of these patients were evaluated. Immunohistochemical study and in situ hybridization (ISH) of EBER1/2 were performed on formalin fixed tissues by tissue chips. The prognosis related factors were analyzed. Results The median age of these 42 EBV⁺ DLBCL patients was 62.5 years. The male-to-female ratio was 2.23∶1.The site of occurrence included lymph node （69.05%） and spleen, stomach, tonsil, nasal cavity and nasopharynx. The mostly common initial clinical presentations were non-specific symptoms, such as lymphadenopathy, splenomegaly, hepatomegaly, fever, and fatigue. Morphologically, the majority (90.48%, 38/42) were pleomorphic subtypes and only 4 cases (9.52%) were simplex subtypes. Immunophenotype showed non-GCB type of DLBCL was predominance (83.33%, 35/42) by Hans classification. The expression of CD30, CD5, BCL-2, P53 and NF-κB/P65 were 52.38%(22/42), 54.76%(23/42), 54.76%(23/42), 87.5%(35/40) and 0%(0/40) respectively. Follow-up data was available in 23(54.76%) patients, 14(60.87%) patients died of the tumor. 5-years overall survival was 16.5%. The median survival time was 40 months. The expression of BCL-2, increased LDH level and starry-sky morphologic character were associated with a poor prognosis. Conclusion EBV positive DLBCL is not uncommon. Most lesions locate in lymph nodes. Pleomorphic histologic subtype is predominant. The tumor has worse prognosis with increased LDH level, starry-sky morphologic character and BCL-2 expression.

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