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

A Clinicopathologic and Prognosis Study of Epstein-Barr Virus Positive Diffuse Large B-cell Lymphoma in Westsouthern China

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。

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 42cases of EBV＋DLBCL in a total 586DLBCL,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 42EBV＋DLBCL patients was 62.5years.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,P53and NF-κB/P65were 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 40months.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-2expression.