CAJ | 학술논문

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-Barr virus,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.
목적:통과대20례피하지막염양T세포림파류(subcutaneous panniculitis-like T-cell lymphoma,SPTL)화19례피부적결외비형NK/T세포림파류적대비연구,가심대2자적인식.방법:종림상병리、면역표기、EB병독(Epstein-Barr virus,EBV)감염화T세포수체(T-cell receptor, TCR)기인중배등다개방면대2자진행비교.결과: 림상표현상2자불역감별,단피부NK/T세포림파류상반피부외파산、예후차;조직학상,SPTL상엄격국한우피하지방조직,이피부NK/T세포림파류이진피위중심형성미만성침윤,상루급피하지방층,경역우견도대편응고성배사、혈관중심성침윤화친표피현상;면역표형상,SPTL상표체βF1、막형CD3、CD8,불표체CD4、CD56,이대부분피부NK/T세포림파류칙표체CD56화세포질CD3ε,부소수표체막형CD3、CD8.CD56、CD3、CD8화βF1적표체차이유통계학의의(P<0.05).SPTL환자중검출 EBER1/2원위잡교양성,이피부NK/T세포림파류100%병례위양성,2자비교차이유통계학의의(P<0.05).SPTL환자중검출TCR-γ기인극륭성중배,이피부NK/T세포림파류환자부유4/18례(22.2%)검출중배,2자지간차이유통계학의의(P<0.05).결론:유무피부외파산,조직학상유무대편응고성배사、혈관중심성침윤화친표피현상,시부표체면역조직화학표기CD56、CD3、CD3ε、CD8화βF1,EB병독원위잡교양성여부,이급TCR-γ극륭성중배검출여부,균가작위SPTL화피부NK/T세포림파류적감별요점.준학감별2자수종합림상、조직병이학、면역표형、EB병독감염화기인중배등결과진행전면분석.
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 gene rearrangement. To make an acute differentiated diagnosis of the two lymphomas, comprehensive analysis is necessary to integrate the results of clinical manifestation, histopathology, immunophenotype, infection of EB virus and gene rearrangement.