皮肤结外鼻型NK/T细胞淋巴瘤7例临床与病理分析

Clinicopathologic Analysis of 7 Cases of Cutaneous Extranodal Natural Killer T Cell Lymphoma,Nasal Type

目的:探讨皮肤结外鼻型NK/T细胞淋巴瘤(extranodal natural killer/T-cell lymphoma,nasal type,ENKTCL-N)的临床特点和组织病理学特征。方法:回顾性分析7例患者的皮疹特点、有无皮肤外损害,并进行临床分期、评价预后及记录实验室检查结果;分析7例病例HE染色的组织病理学特点及免疫组化结果。结果:7例患者中男5例,女2例,平均年龄(41.7±22.7)岁,平均病程31.4(2～48)个月。7例患者中6例(85.7%)患者皮损累及四肢,全部患者皮疹均具有多发性和多形性的特点及慢性溃疡的表现,溃疡大小不一,圆形或椭圆形、深在、边缘整齐;在发现皮肤损害时,6例(85.7%)患者同时有皮肤外损害;7例患者中,4例(57.1%)死亡,3例(42.9%)带瘤生存。6例(85.7%)患者外周血淋巴细胞亚群异常,亦有6例(85.7%)患者EB病毒抗体阳性。皮损处组织病理表现为致密的肿瘤细胞浸润真皮和皮下脂肪,并侵及血管;瘤细胞多数为中等大小,常见核分裂象。免疫组化显示全部患者异型的T细胞相关标志CD45RO/UCHL-1阳性及EB病毒原位杂交阳性,CD56阳性者3例(42.9%)。结论:本病呈多形性、多发性,并以慢性溃疡为主的皮损可能是发现ENKTCL-N的线索,外周血淋巴细胞亚群异常可能对疾病诊断有提示作用,而皮肤组织病理见大量异形淋巴细胞及侵血管现象对诊断很有价值,最后确诊需要结合病理组织、免疫组化。

Objective： To investigate the clinicopathologic features of cutaneous extranodal natural killer （NK）/T cell lymphoma, nasal type （ ENKTCL-N ）. Methods： Seven cases of extranodal NK/T cell lymphomas were retrospectively studied. The cutaneous manifestations, clinical data, laboratory examinations, and pathological data were analyzed. Results： Five males and two female patients were recruited in this study. The average patient age was 41.7 years. The skin lesions in these cases were multiple and pleomorphic. The skin lesions of six patients （ 85.7% ） involved limbs. Chronic ulcers were prominently present in all patients. The ulcers varied in size and shape, round, elliptical, deep, and with regular edge. Extracutaneous lesions were found in six cases （ 85.7% ）. Four of the patients （ 57.1% ） died and the remaining three cases （ 42.9% ） survived with the disease. Six patients （ 85.7% ） had abnormalities in the lymphocyte subsets and six cases were positive for Epstein - Barr virus antibodies in the peripheral blood. Histopathology of the skin lesions showed dense tumor cell infiltration into the dermis and subcutaneous fat. The tumor cells were of medium size with nuclear mitoschisis. The results of the immunohistochemistry showed the tumor cells were positive for CD45RO/UCHL-1 and EBER in seven cases and CD56 in three cases. Conclusion： Multiple and pleomorphic skin lesions and chronic ulcers could be clues for diagnosis. Abnormalities in the lymphocyte subsets in peripheral blood can help diagnose ENKTCL-N. Histopathologic and immunohistochemical data are needed for a definitive diagnosis.