结外鼻型NK/T细胞淋巴瘤28例临床病理分析  

Extranodal NK/T-cell lymphoma,nasal type: a clinicopathological analysis of 28 cases

作　　者：陈定宝[1] 王颖[1] 宋秋静[1] 沈丹华[1] 

机构地区：[1]北京大学人民医院病理科,北京100044

出　　处：《临床与实验病理学杂志》2015年第4期404-408,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨结外鼻型NK/T细胞淋巴瘤(extranodal natural killer/T-cell lymphoma,nasal type,EN-NK/TCL)的临床病理学特征、诊断及鉴别诊断。方法应用免疫组化、EBER原位杂交技术检测28例EN-NK/TCL,依据WHO(2008)淋巴造血组织肿瘤分类进行临床病理学分析。结果 28例EN-NK/TCL中,男性13例,女性15例,男女之比为1∶1.2,平均年龄46岁,中位年龄44.5岁。18例发生于鼻腔,5例发生于皮肤,扁桃体、上腭、舌根、肾上腺、胃各1例。临床主要表现为鼻塞,皮肤病变可表现为皮疹、溃疡、斑块或结节形肿物。可伴有B症状、淋巴结肿大,可累及多个部位。肿瘤细胞为小、中、大淋巴细胞或呈混合型,常见血管中心性或血管破坏的生长方式及坏死,多伴有炎细胞浸润。免疫表型:T细胞标志物(CD3ε最敏感)、CD56及细胞毒性标志物均阳性,原位杂交EBER呈阳性。10例获得随访,其中1例死亡。结论 EN-NK/TCL好发于上呼吸道,其次是皮肤。结合形态学、免疫表型及EBER原位杂交结果可确诊。Purpose To study the clinicopathologic features, diagnosis and differential diagnosis of extranodal NK/T-cell lymphoma, nasal type （EN-NK/TCL）. Methods Twenty-eight cases of EN-NK/TCL were analyzed according to WHO classification of tumors of haematopoietic and lymphoid tissues, the cases were studied by microscopy, immunohistochemistry and in situ hybridization. Results In 28 cases, the male to female ratio was 1 ： 1.2. The mean age was 46 years, with the median of 44. 5. Eighteen cases involved nasal cavity, 5 cases of skin, and 1 case of tonsil, upper palate, root of tongue, adrenal glands and gastric, respectively. The main presentation was nasal obstruction in cases with nasal involvement, and the cases with skin involvement present with rash, ulcer, plaque or nodular mass. Patients might be accompanied by B-type symptoms, lymphoadenopathy or with muhiple sites involvement. The tumor cells were small, medium, large-sized, or mixed, and angiocentric and angiodestructive growth pattern was frequently pres- ent. Most cases were mixed with inflammatory cells infiltration. The typical immunophenotype was T-cell （the most sensitive, CD3e） , CD56 and cytotoxic markers. In situ hybridization for EBER was positive. Ten cases were followed up, and 1 case died. Conclusions The most common site of EN-NK/TCL is nasal cayity, followed by skin. EN-NK/TCL can be definitely diagnosed by integration of pathological morphology, immunophenotype and in situ hybridization of EBER.

关 键 词：淋巴瘤 T细胞淋巴瘤 NK/T细胞淋巴瘤 EB病毒 细胞毒性颗粒 

分 类 号：R733.4[医药卫生—肿瘤]