脾复合性淋巴瘤临床病理分析

Composite lymphoma of spleen:a clinicopathological analysis

目的:探讨脾脏复合性淋巴瘤的临床病理特征、诊断要点及鉴别诊断,提高对复合性淋巴瘤的诊治水平。方法:对1例发生于脾脏的复合性T与B细胞淋巴瘤病例进行临床、组织学、免疫组化、电镜和基因重排等方法观察并结合文献分析。结果:患者男性,36岁。临床表现为上腹不适,影像学检查提示脾脏占位性病变。组织学显示瘤组织在脾脏内弥漫性排列,瘤细胞呈圆形、卵圆形及不规则的梭形,胞浆丰富,核染色质粗糙,可见核分裂像,部分核呈空泡状。瘤细胞表达B细胞免疫表型:CD20、CD79、Bcl-2(+),CD10、CD23(-);表达T细胞免疫表型:CD3、CD8、CD45RO(+);CD4(-)。S-100、CD1a、CD21、CD30、ER、HMB45、Act、Fascin、ALK均(-)。基因重排结果显示:免疫球蛋白基因及TCR受体基因克隆性重排均为阳性。电镜示瘤细胞的胞质内游离核糖体丰富,有少量线粒体、粗面内质网和溶酶体。结论:复合性淋巴瘤临床发生率较低,而本例复合性T与B细胞淋巴瘤更为罕见,诊断比较困难。其诊断和鉴别诊断依赖于病理组织学、免疫表型分子和分子遗传学并结合临床特点综合分析。

Objective ：To study the clinical pathological features, main diagnostic points and differential diagnosis of composite lymphoma of spleen. To improve the diagnose and management of composite lymphoma. Methods： A case of composite lymphoma of spleen was studied by light microscopy , immunohistochemical staining, electron mi- croscope and generearrangement. Results： Histologically, the diffused and disorded neoplastic cells were round, oval or irregular spindle contained hyperchromatic nuclei, subtle nuclear pleomorphism, mitotic figures. Nuclei were partly pale - stained with nucleolus and may be foamy. Immunohistochemically tumor cells were positive for Bcl - 2, CD3, CD8,CD15,CD20,CD21,CD35, CD43, CD45, CD45RO, CD68, CD117, CD163, clusterin, CNA4.2, HLA - Dr, HLA -Dp, Lysozyme, Vimentin, but negative for S - 100, CDla, CD4, CD30, CD23, ER, HMB45, DES, ACT, Fascin, ALK. Conclusion： As composite lymphoma is a rare tumor, easily neglected or misdiagnosed. The diagnosis and dif- ferential diagnosis rely mainly on the general analysis of histopathologic changes, immunohistochemical staining, elec- tron microscope and generearrangement.