原发皮肤结外NK/T细胞淋巴瘤-鼻型临床组织病理、免疫表型及基因型分析  

Clinicopathological features,immunohistochemical and genetic phenotypes of primary cutaneous extranodal NK/T cell lymphoma-nasal type

作　　者：张爽[1] 阿迪拉.斯依提 农琳[1] 李阳[1] 涂平[3] 陈喜雪[3] 朱学骏[3] 李挺[1] 

机构地区：[1]北京大学第一医院病理科,北京100034 [2]新疆医科大学肿瘤医院病理科,乌鲁木齐830011 [3]北京大学第一医院皮肤科,北京100034

出　　处：《临床皮肤科杂志》2010年第11期684-688,共5页Journal of Clinical Dermatology

摘　　要：目的:探讨原发皮肤结外NK/T细胞淋巴瘤-鼻型(EN-NK/T-NT)的临床、组织病理学、免疫表型及基因型特点。方法:对2例原发皮肤EN-NK/T-NT进行临床特征、组织病理学形态和免疫组化分析,以及EB(Ebstein-Barr)病毒(EBV)原位杂交及T细胞受体γ基因PCR重排检测。结果:2例患者发病均表现为皮肤症状,双下肢皮肤多发浸润性红斑伴溃疡,无中面部症状。组织病理均示中至大型异形淋巴细胞弥漫浸润真皮和皮下组织,肿瘤细胞核不规则,核仁不明显,胞质中等量,可见血管壁浸润,凋亡及坏死明显。免疫组化肿瘤细胞CD3ε、CD56、细胞毒蛋白、表达于T细胞的T-box(T-bet)及erythroblastosis virus E26oncogene homolog(ETS)-1阳性,皮肤淋巴细胞相关抗原(CLA)例1阴性、例2阳性;EBV强阳性;T细胞受体γ基因重排呈胚系构型。结论:原发皮肤EN-NK/T-NT与原发鼻EN-NK/T-NT组织病理学及相关分子生物学特点一致,其发生与发展转归值得深入研究。Objective： To analyze the clinical and histopathological features, immunophenotypes and genetic phenotypes of the cases of primary cutaneous extranodal NK/T cell lymphoma-nasal type （EN-NK/T-NT）. Methods： The clinical features and histological changes of two cases, a 60-year-old woman and a 75-yearold man, of primary EN-NK/T-NT were analyzed. The immunochemistry assay, in situ hybridization of EBV, and PCR for TCRγ rearrangement was also performed. Results： At the onset of the disease, both patients presented with skin lesions, multiple infiltrated erythema and ulcers on the legs, with no lesions on the midface. The histopathology of the skin lesion showed diffuse infiltration of moderate to large tumor ceils in the dermis and subcutaneous tissue, with irregular dysplastic nuclei. The vessel wall was infiltrated with obvious apoptosis and necrosis. The tumor cells were positively stained for CD3a, CD56, cytotoxic markers, T-bet and ETS-1 in both cases, and positively stained for CLA in one case. In situ hybridization showed that EBV existed in all tumor cells. TCRT gene rearrangements were presented in germline configuration. Conclusions： The two cases reported show the same histopathologic features, immunophenotype and genetic phenotype as usual cases of EN-NK/T-NT, the carcinogenic mechanisms of which needs further study.

关 键 词：皮肤 淋巴瘤 结外NK/T细胞 

分 类 号：R739.5[医药卫生—肿瘤]