Cao Meng-Shu, Cai Hou-Rong, Yin Hong-Lin, Zhang De-Ping, Xiao Yong-Long, Cao Min, Dai Ling-Juan, Hou Jie
Department of Respiratory Medicine, The Affiliated Drum Tower Hospital, Nanjing University Medical College, Nanjing 210008, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2008 Feb;31(2):120-4.
To describe the clinical and pathological features of primary NK/T cell lymphoma of the lung.
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.
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).
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.
描述肺原发性NK/T细胞淋巴瘤的临床和病理特征。
报告2例肺原发性NK/T细胞淋巴瘤病例,并结合3例文献复习,探讨该疾病的临床、影像学及病理特征。
多数患者表现为发热、咳嗽和呼吸困难,抗生素治疗无效。影像学表现包括孤立或多发结节及实变、单侧或双侧胸腔积液(4/5),无肺门或纵隔淋巴结肿大。5例患者中3例EB病毒阳性。组织病理学显示大量异常淋巴细胞浸润,呈血管中心性,伴有明显组织坏死和血管破坏。免疫表型分析显示CD56(+)、CD3(+)、穿孔素(+)、T细胞胞浆内抗原-1(+)和/或颗粒酶B(+),但CD20(-)。多数患者在半年内死于呼吸衰竭(4/5)。
肺原发性NK/T细胞淋巴瘤罕见,当患者出现肺部阴影、对抗生素治疗无效的发热、白细胞减少及乳酸脱氢酶升高时应考虑该病。