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[原发性纵隔大B细胞淋巴瘤:27例临床病理研究]

[Primary mediastinal large B-cell lymphoma: a clinicopathologic study of 27 cases].

作者信息

Shi Q Y, Feng X, Chen H, Ma H H, Lu Z F, Shi Q L, Zhou X J, Shen Q

机构信息

Department of Pathology, Nanjing General Hospital of PLA, Nanjing Medical University, Nanjing 210002, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2017 Sep 8;46(9):607-612. doi: 10.3760/cma.j.issn.0529-5807.2017.09.004.

Abstract

To study the clinicopathologic characteristics and diagnostic criteria of primary mediastinal B-cell lymphoma (PMBL), and to distinguish PMBL from classic Hodgkin lymphoma(CHL) and systemic diffuse large B-cell lymphoma(DLBCL). The clinical features, histologic findings, results of immunohistochemical study and prgnosis in 27 PMBL cases were analyzed, with review of literature. The age of patients ranged from 19 to 82 years (median age 34 years). All cases were located in the mediastinum and frequently accompanied by superior vein cava syndrome. Histologically, the tumor cells were pleomorphic and diffusely distributed. Clear cytoplasm and spindle tumor cells were seen in some cases. Varying amount of sclerosing stroma with collagen deposition was seen.Immunohistochemical study showed that the tumor cells were positive for CD20(100%, 27/27), CD30 (64.0%, 16/25), CD23 (77.3%, 17/22) and p63 (16/19). Clonal B cell gene rearrangement was seen. PMBL is a subtype of diffuse large B-cell lymphoma with various histomorphology. Immunohistochemistry can help to confirm the diagnosis, and the prognosis is better than diffuse large B cell lymphoma, not otherwise specified.

摘要

研究原发性纵隔B细胞淋巴瘤(PMBL)的临床病理特征及诊断标准,以鉴别PMBL与经典型霍奇金淋巴瘤(CHL)及系统性弥漫大B细胞淋巴瘤(DLBCL)。分析27例PMBL患者的临床特征、组织学表现、免疫组化研究结果及预后,并复习相关文献。患者年龄19至82岁(中位年龄34岁)。所有病例均位于纵隔,常伴有上腔静脉综合征。组织学上,肿瘤细胞多形性,呈弥漫性分布。部分病例可见透明细胞质及梭形肿瘤细胞。可见不同量伴有胶原沉积的硬化性间质。免疫组化研究显示肿瘤细胞CD20阳性(100%,27/27)、CD30阳性(64.0%,16/25)、CD23阳性(77.3%,17/22)及p63阳性(16/19)。可见克隆性B细胞基因重排。PMBL是弥漫大B细胞淋巴瘤的一种亚型,具有多种组织形态学表现。免疫组化有助于确诊,其预后优于未另行特指的弥漫大B细胞淋巴瘤。

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