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目的 探讨表现为间质性肺炎的肺血管内大B细胞淋巴瘤(IVLBCL)的临床特征、影像学特点及预后.方法 回顾性分析南京大学医学院附属鼓楼医院2010年3月至2012年5月收治的2例表现为间质性肺炎的肺IVLBCL患者的临床资料.以“肺血管内淋巴瘤”为检索词在万方数据库、中国知网数据库,以英文检索词“Pulmonary intravascular lymphoma”在PubMed数据库中检索大B细胞淋巴瘤的相关文献,检索时间为1980年1月至2015年6月,分析其临床特征、肺部影像学特点以及预后.结果 例1女,69岁,胸部高分辨率CT表现为磨玻璃影、小叶间隔增厚及斑片状实变影,经支气管镜肺活检确诊.例2男,70岁,胸部高分辨率CT表现为弥漫性磨玻璃影,经外科胸腔镜肺活检确诊.2例患者临床主要表现为咳嗽、咳痰、气喘及发热等,血乳酸脱氢酶升高;肺活检病理特点为肺组织毛细血管及小血管内见多量异形淋巴细胞,异型淋巴细胞表达CD20.文献检索后经过筛选剔除,共检索出相关文献19篇,均为个案报道,共报道22例.临床主要表现为发热、咳嗽及气喘;影像学表现为磨玻璃影、小结节影、实变影及小叶间隔增厚等.结论肺IVLBCL临床表现无特异性,进展迅速.故当患者胸部高分辨率CT表现为间质性肺炎时,需考虑肺IVLBCL的可能,并尽快完善病理学诊断,以早期治疗,改善预后.

Objective To investigate the clinical,radiographic characteristics and prognosis of pulmonary intravascular large B-cell lymphoma(IVLBCL) manifesting as interstitial pneumonias on HRCT.Methods A retrospective analysis was carried out on clinical data of 2 patients with pulmonary IVLBCL admitted to the Affiliated Drum Tower Hospital of Nanjing University from March 2010 to May 2012.A literature research was performed with "pulmonary intravascular lymphoma" as the key word in Wanfang Database,China Knowledge Resource Integrated Database and Pubmed.The time interval was from January 1980 to June 2015.Related articles of pulmonary IVLBCL were retrieved and the clinical,radiographic characteristics and prognosis were analyzed.Results The first patient was a 69 year-old female presenting with ground-glass opacities,interlobular septal thickening and patchy consolidation on HRCT,for whom the diagnosis was confirmed by transbronchial lung biopsy (TBLB).The second patient was a 70 year-old male presenting with diffuse ground-glass opacities on HRCT,and the diagnosis was made by pathology on videoassisted thoracoscopic biopsy.The 2 patients all presented with dyspnoea,cough,fever and elevated lactate dehydrogenase(LDH).The pathological study of lung biopsy specimen demonstrated invasion of atypical lymphocytes into small vessels and capillaries.The tumor cells were positive for CD20.Literature review found 19 articles,all case reports with a total of 22 patients.Conclusions The clinical manifestation of pulmonary IVLBCL was nonspecific and the disease progressed rapidly.For patients with interstitial pneumonias on HRCT,pulmonary IVLBCL needed to be considered as a differential diagnosis and pathological studies should be obtained as soon as possible,so that better prognosis could be archived through early intervention.