皮下脂膜炎样T细胞淋巴瘤与皮肤的结外鼻型NK/T细胞淋巴瘤的对比研究  

A comparative study between subcutaneous panniculitis-like T-cell lymphoma and cutaneous extranodal nasal-type NK/T cell lymphoma

作　　者：杨翰君[1] 万川[1,2] 王婷婷[1] 徐晨[1] 刘卫平[3] 李甘地[3] 王琳[1] 

机构地区：[1]四川大学华西医院皮肤性病科,成都610041 [2]江西省皮肤病专科医院,南昌330001 [3]四川大学华西医院病理科,成都610041

出　　处：《肿瘤》2010年第2期143-147,共5页Tumor

基　　金：2005年四川大学华西医院留学回国人员科研启动基金资助项目(编号:136050122)

摘　　要：目的:通过对20例皮下脂膜炎样T细胞淋巴瘤(subcutaneous panniculitis-like T-cell lymphoma,SPTL)和19例皮肤的结外鼻型NK/T细胞淋巴瘤的对比研究,加深对2者的认识。方法:从临床病理、免疫标记、EB病毒(Epstein-Barrvirus,EBV)感染和T细胞受体(T-cell receptor,TCR)基因重排等多个方面对2者进行比较。结果:临床表现上2者不易鉴别,但皮肤NK/T细胞淋巴瘤常伴皮肤外播散、预后差;组织学上,SPTL常严格局限于皮下脂肪组织,而皮肤NK/T细胞淋巴瘤以真皮为中心形成弥漫性浸润,常累及皮下脂肪层,更易于见到大片凝固性坏死、血管中心性浸润和亲表皮现象;免疫表型上,SPTL常表达βF1、膜型CD3、CD8,不表达CD4、CD56,而大部分皮肤NK/T细胞淋巴瘤则表达CD56和细胞质CD3ε,仅少数表达膜型CD3、CD8。CD56、CD3、CD8和βF1的表达差异有统计学意义(P<0.05)。SPTL患者中检出EBER1/2原位杂交阳性,而皮肤NK/T细胞淋巴瘤100%病例为阳性,2者比较差异有统计学意义(P<0.05)。SPTL患者中检出TCR-γ基因克隆性重排,而皮肤NK/T细胞淋巴瘤患者仅有4/18例(22.2%)检出重排,2者之间差异有统计学意义(P<0.05)。结论:有无皮肤外播散,组织学上有无大片凝固性坏死、血管中心性浸润和亲表皮现象,是否表达免疫组织化学标记CD56、CD3、CD3ε、CD8和βF1,EB病毒原位杂交阳性与否,以及TCR-γ克隆性重排检出与否,均可作为SPTL和皮肤NK/T细胞淋巴瘤的鉴别要点。准确鉴别2者需综合临床、组织病理学、免疫表型、EB病毒感染和基因重排等结果进行全面分析。Objective：To compare the difference between 20 cases of subcutaneous panniculitis-like T-cell lymphoma （SPTL） and 19 cases of cutaneous extra-nodal nasal-type NK/T-cell lymphoma （cutaneous NK/T-cell lymphoma）. Methods：The two types of lymphoma were compared in clinical pathology,immunological marker,Epstein-Barr （EB） virus infection,and T-cell receptor （TCR） gene rearrangement. Results：Differentiated diagnosis of the two types of lymphomas was not easy based on their clinical manifestations,but the cutaneous NK/T-cell lymphoma was always followed by extracutaneous dissemination and had a poor prognosis. Histopathologically,SPTL was usually limited in subcutaneous fatty tissues while the cutaneous NK/T-cell lymphoma showed diffused infiltration around the dermis and it often infiltrated into the subcutaneous fat tissues. Coagulation necrosis,angiocentric infiltration and epidermotropism were often observed in cutaneous NK/T-cell lymphoma. When compared with immunophenotypes,SPTL often expressed βF1,membrane CD3 and CD8 but did not express CD4 and CD56,while most of the cutaneous NK/T-cell lymphomas expressed CD56 and cytoplasm CD3ε and only a few cases expressed CD3 and CD8. The differences in the expression of CD56,CD3,CD8,and βF1 were significant between the two types of lymphomas（P〈0.05）. The positive rate of EBER1/2 was 25% （5/20） in SPTL while it was 100% in cutaneous NK/T-cell lymphoma. The difference was statistically significant （P〈0.05）. Monoclonal TCR-γ gene rearrangement was found in 16 out of 20 cases of SPTL （80%） but only in 4 of 18 cases in the cutaneous NK/T-cell lymphoma （22.2%）. The difference was significant（P〈0.05）. Conclusion：The key points to distinguish the two lymphomas are （1） extracutaneous dissemination,coagulation necrosis,angiocentric infiltration and epidermotropism; （2） the expressions of CD56,CD3,CD3ε,CD8,and βF1; （3） the positivity of in situ hybridization of EB virus; （4） detection of the monoclonal TCR-γ

关 键 词：皮肤肿瘤 淋巴瘤 T细胞 皮肤 免疫组织化学 对比研究 

分 类 号：R733.4[医药卫生—肿瘤]