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目的探讨纵隔结节硬化型霍奇金淋巴瘤(NSHL)的临床病理学特征,提高对该疾病的认识。方法收集2003年-2012年纵隔NSHL手术切除3例患者的病历资料。对纵隔NSHL病理标本采用形态学、免疫表型、T细胞受体(TCR)γ和Ig H基因重排及EB病毒编码小RNA(EBER)原位杂交等方法进行检测和分析。结果纵隔NSHL的病理形态为胶原纤维显著增生成束状并分割淋巴组织形成多个结节,肿瘤细胞边界清楚、细胞质空亮,单核或多个小核仁,结节背景有炎细胞浸润;肿瘤细胞均表达CD15、CD30及PAX-5,部分肿瘤细胞表达CD20,均不表达间变性淋巴瘤激酶、CD45、细胞角蛋白、CD79α、S-100等标记,Ki-67(+,>40%);3例肿瘤细胞均无TCRγ、Ig H基因重排;EBER原位杂交均为阴性。结论纵隔可发生多种淋巴瘤,NSHL是其中一种,掌握其独特的病理形态学特点及免疫表型对该疾病的诊断、鉴别诊断及治疗非常重要。
Objective To investigate the clinicopathological features of mediastinal nodal cirrhosis Hodgkin’s lymphoma (NSHL) and to improve their understanding of the disease. Methods The data of 3 cases of surgical resection of mediastinal NSHL from 2003 to 2012 were collected. The pathological specimens of mediastinal NSHL were detected and analyzed by morphology, immunophenotype, TCR γ and Ig H gene rearrangement and EB virus-encoded small RNA (EBER) in situ hybridization. Results The pathologic morphology of mediastinal NSHL was that collagen fibers proliferated into bundles and segmented lymphoid tissues to form multiple nodules. The tumor cells had a clear boundary, empty cytoplasm, mononuclear or multiple small nucleoli and inflammatory cell infiltration in the nodule. The tumor cells expressed CD15, CD30 and PAX-5, while some of the tumor cells expressed CD20, but none of them expressed markers such as anaplastic lymphoma kinase, CD45, cytokeratin, CD79α, S-100, ). No TCRγ and Ig H gene rearrangements were found in all the three tumor cells. All the EBER in situ hybridization were negative. Conclusion There are many kinds of lymphomas in the mediastinum. NSHL is one of them. It is very important to know its unique pathomorphological features and immunophenotypes in the diagnosis, differential diagnosis and treatment of this disease.