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目的:探讨原发性纵隔大B细胞淋巴瘤(primary mediastinal large B-cell lymphoma,PMBL)的临床病理学特点及诊断要点。方法:收集2010年9月~2014年12月病理确诊为PMBL的病例,对其对PMBL进行临床特点、病理形态学及免疫组织化学观察分析,并复习相关文献。结果:3例PMBL2例为男性,1例为女性,3例均侵犯邻近器官,2例伴颈部或锁骨上淋巴结受累,1例椎体受累(C7-T4)。镜下见不同程度的纤维化,瘤细胞呈巢状或弥漫浸润,瘤细胞胞质空亮丰富,细胞核圆形或卵圆形,其中1例可见坏死。免疫组织化学均表达CD20、CD79a、CD23、bcl2、CD23,其中2例表达CD30,均不表达CD3、CD5。随访3例均生存,化疗后1例获得CR,2例获得PR。结论:纵隔原发弥漫大B细胞淋巴瘤很少见,形态变化多端,容易引起误诊。提高对PMBL的认识,对避免误诊是至关重要的。
Objective: To investigate the clinicopathological features and diagnosis of primary mediastinal large B-cell lymphoma (PMBL). Methods: The cases with pathologically confirmed PMBL from September 2010 to December 2014 were collected. The clinical features, pathomorphology and immunohistochemistry of PMBL were observed and reviewed. Results: Three cases of PMBL were male and one case of female. All three cases had violations of adjacent organs, two cases had neck or supraclavicular lymph node involvement, and one case had vertebral body involvement (C7-T4). Microscopically, fibrosis was seen in varying degrees. The neoplastic cells were nested or diffusely infiltrated. The cytoplasm of the tumor cells were brightly empty and the nucleus was round or oval. One of them showed necrosis. Immunohistochemistry showed that CD20, CD79a, CD23, bcl2, CD23, of which 2 cases expressed CD30, did not express CD3, CD5. Three patients were followed up for survival. One patient achieved CR after chemotherapy and two received PR. CONCLUSIONS: Primary mediastinal diffuse large B-cell lymphoma is uncommon and has multiple morphological changes that can easily lead to misdiagnosis. Increasing awareness of PMBL is crucial to avoiding misdiagnosis.