累及肺部的血管内淋巴瘤病一例并文献复习

目的 报道1例以肺部症状和体征为主要表现的血管内淋巴瘤病.方法 对2008年3月入住北京协和医院的36岁男性患者进行临床、影像学、病理组织学检查,明确诊断后行环磷酰胺+表柔比星+长春新碱+泼尼松(CHOP)方案化疗.从文献中检索到14例以肺部症状和体征为主要表现,无皮肤和中枢神经系统临床表现的血管内淋巴瘤病.对15例的临床、影像学、病理组织学特点、治疗及预后进行总结.结果 患者以发热和肺部多发结节就诊,经开胸肺活检病理证实为T细胞性血管内淋巴瘤病,虽经CHOP方案化疗,但病情不断加重,诊断20 d后死亡.总结以肺部及呼吸系统症状和体征为主要表现的血管内淋巴瘤病主要见于老年人,年龄为36～79岁,中位年龄59岁;男11例,女4例;主要症状为气短(10/15)、发热(9/15)、咳嗽(5/15)、体重减轻(5/15)和乏力(2/15)等;肺功能检查以弥散功能障碍为主,可伴有不同程度的阻塞性通气功能障碍,1例有肺动脉高压,8例的血清乳酸脱氢酶升高.胸部CT示双肺弥漫性条索状阴影和(或)多发结节影,部分结节呈楔形,位于胸膜下,常伴有肺支气管血管束增粗.10例为B细胞性,3例为T细胞性,2例未区分细胞类型.有随访资料的B细胞性血管内淋巴瘤病6例,均行CHOP方案化疗,4例存活,2例死于呼吸衰竭;3例T细胞性血管内淋巴瘤病的预后均较差,经CHOP方案化疗的效果不佳,分别于诊断后20 d和3个月死亡.结论 以肺部症状和体征为主要表现的血管内淋巴瘤病较为少见,其临床症状无特异性,肺功能检查主要为弥散功能障碍,常伴有血清乳酸脱氢酶升高,胸部CT示双肺弥漫性条索状阴影和(或)多发结节影,部分结节呈楔形,位于胸膜下,常伴有肺支气管血管束增粗,纵隔肺门淋巴结无肿大.经支气管肺活检或开胸肺活检病理检查可明确诊断.

Intravascular Iymphomatosis presenting in the lung

Objective To investigate the clinical, radiographic and pathological characteristics of intravascular lymphomatosis primarily manifested in the lung, without skin and central nervous system involvements. Methods A case of T cell intravascular lymphomatosis presenting with fever and multiple pulmonary shadows on chest radiograph was described and 14 similar cases reported in the English literature were reviewed. Results We described a case of T cell intravascular lymphomatosis, who was a 36 year old man,complained of fever and multiple pulmonary shadows on chest radiograph and admired to Peking Union Medical College Hospital in march, 2008. Open lung biopsy showed features characteristic of intravascular lympbomatosis. He received CHOP chemotherapy, but died 20 days after diagnosis. Most cases of intravascular lymphomatosis primarily manifested in the lung occurred in older patients, ranging from 36 to 79 years of age (mean 59 years), with a male predominance (M:F=11:4). The chief complaints were dyspnoea (10/15) , cough (9/15), fever (5/15) and weight loss (5/15). Pulmonary function tests usually revealed some degree of decreased diffusing capacity. Eight cases had high serum lactate dehydrogenase levels. Chest CT scan showed multiple reticular or/and nodular density, Immunophenotypically,10 cases were B cell lineage, 3 cases were T cell lineage. Six cases of B cell intravascular lymphomatosis were followed, of whom 4 and were alive, and 2 died of respiratory failure. Three cases of T cell intravascular lymphomatosis showed poor prognosis, both of whom died of respiratory failure. Conclusions Intravascular lymphomatosis primarily manifested in the lung is a rare malignant disease. Its pulmonary symptoms were nonspecific, and pulmonary function tests and chest CT scan manifested as those of interstitial pneumonia or pulmonary infection. It can be pathologically diagnosed through transbronchiai lung biopsy or surgical lung biopsy.