CAJ | 학술논문

目的提高对原发性肺自然杀伤(natural killer,NK)/T细胞淋巴瘤的诊断和鉴别.方法报道2006年我院收治的2例原发性肺NK/T细胞淋巴瘤患者,复习国内外文献报道的3例患者临床及实验室检查特征.结果患者临床表现为发热、咳嗽、呼吸困难等,抗生素治疗无效.胸部影像学表现为单发或多发的团块状、斑片状实变影,可出现单侧或双侧胸腔积液(4/5),无肺门和纵隔淋巴结肿大.多数患者(3/5)EB病毒(+).肺组织活检病理学表现为大量异常淋巴细胞浸润,肿瘤细胞呈血管中心性生长,并伴有明显的组织坏死和血管破坏;肿瘤细胞免疫表型为CD56(+)、CD3(+),穿孔素(+)、T淋巴细胞胞质内抗原-1(+)和(或)颗粒酶(+),CD20(-).大部分患者(4/5)在半年内由于呼吸衰竭死亡.结论原发性肺NK/T细胞淋巴瘤罕见,且临床表现为发热伴肺部阴影且疑诊肺炎的患者,若同时合并白细胞减少伴乳酸脱氢酶明显升高,在鉴别诊断时应考虑原发性肺NK/T细胞淋巴瘤.
목적 제고대원발성폐자연살상(natural killer,NK)/T세포림파류적진단화감별.방법 보도2006년아원수치적2례원발성폐NK/T세포림파류환자,복습국내외문헌보도적3례환자림상급실험실검사특정.결과 환자림상표현위발열、해수、호흡곤난등,항생소치료무효.흉부영상학표현위단발혹다발적단괴상、반편상실변영,가출현단측혹쌍측흉강적액(4/5),무폐문화종격림파결종대.다수환자(3/5)EB병독(+).폐조직활검병이학표현위대량이상림파세포침윤,종류세포정혈관중심성생장,병반유명현적조직배사화혈관파배;종류세포면역표형위CD56(+)、CD3(+),천공소(+)、T림파세포포질내항원-1(+)화(혹)과립매(+),CD20(-).대부분환자(4/5)재반년내유우호흡쇠갈사망.결론 원발성폐NK/T세포림파류한견,차림상표현위발열반폐부음영차의진폐염적환자,약동시합병백세포감소반유산탈경매명현승고,재감별진단시응고필원발성폐NK/T세포림파류.
Objective To describe the clinical and pathological features of primary NK/T cell lymphoma of the lung.Methods Two cases of primary NK/T cell lymphoma of the lung were repoaed,and the clinical,radiological and pathological characteristics of the disease were discussed with literature review of 3 cases.Results Most patients presented with fever,cough and dyspnea,and antibiotics were ineffective.Radiographic findings included solitary or multiple nodules and consolidation,unilateral orbilateral pleural effusions(4/5),without hilar or mediastinal adenopathy.Ebstein-Barr virus was positive in cases patients(3/5).Histopathology revealed a great deal of abnormal lymphocyte infiltration,which were angio-centric with marked tissue putrescence and angio-destruction.Immunophenotyping showed CD56(+),CD3(+),perform(+),T-cell intracytoplasmic antigen-1(+)and/or GranB(+),but CD20(-).Most patients died of respiratory failure in half a year(4/5).Conclusion Primary NK/T cell lymphoma of the lung is rare,but should be considered when patients present with lung shadows and fever non-responsive to antibiotics,decreased WBC and increased LDH.