CAJ | 학술논문

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。
목적：탐토결외비형NK/T세포림파류( extranodal natural killer/T-cell lymphoma, nasal type, EN-NK/TCL)적림상병이학특정、진단급감별진단。방법응용면역조화、EBER원위잡교기술검측28례EN-NK/TCL,의거WHO(2008)림파조혈조직종류분류진행림상병이학분석。결과28례EN-NK/TCL중,남성13례,녀성15례,남녀지비위1∶1．2,평균년령46세,중위년령44．5세。18례발생우비강,5례발생우피부,편도체、상악、설근、신상선、위각1례。림상주요표현위비새,피부병변가표현위피진、궤양、반괴혹결절형종물。가반유B증상、림파결종대,가루급다개부위。종류세포위소、중、대림파세포혹정혼합형,상견혈관중심성혹혈관파배적생장방식급배사,다반유염세포침윤。면역표형：T세포표지물( CD3ε최민감)、CD56급세포독성표지물균양성,원위잡교EBER정양성。10례획득수방,기중1례사망。결론 EN-NK/TCL호발우상호흡도,기차시피부。결합형태학、면역표형급EBER원위잡교결과가학진。
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 multiple 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, CD3ε) , 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 cavity, followed by skin. EN-NK/TCL can be definitely diagnosed by integration of pathological morphology, immunophenotype and in situ hybridization of EBER.