原发性肺自然杀伤／T细胞淋巴瘤二例临床分析

Primary natural kmer/T cell lymphoma of the lung：two cases report and clinical analysis

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（＋）、CD，（＋），穿孔素（＋）、T淋巴细胞胞质内抗原-1（＋）和（或）颗粒酶（＋），CD加（-）。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。

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 reported, 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.