CAJ | 학술논문

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.
목적 보도1례이폐부증상화체정위주요표현적혈관내림파류병.방법 대2008년3월입주북경협화의원적36세남성환자진행림상、영상학、병리조직학검사,명학진단후행배린선알+표유비성+장춘신감+발니송(CHOP)방안화료.종문헌중검색도14례이폐부증상화체정위주요표현,무피부화중추신경계통림상표현적혈관내림파류병.대15례적림상、영상학、병리조직학특점、치료급예후진행총결.결과 환자이발열화폐부다발결절취진,경개흉폐활검병리증실위T세포성혈관내림파류병,수경CHOP방안화료,단병정불단가중,진단20 d후사망.총결이폐부급호흡계통증상화체정위주요표현적혈관내림파류병주요견우노년인,년령위36～79세,중위년령59세;남11례,녀4례;주요증상위기단(10/15)、발열(9/15)、해수(5/15)、체중감경(5/15)화핍력(2/15)등;폐공능검사이미산공능장애위주,가반유불동정도적조새성통기공능장애,1례유폐동맥고압,8례적혈청유산탈경매승고.흉부CT시쌍폐미만성조색상음영화(혹)다발결절영,부분결절정설형,위우흉막하,상반유폐지기관혈관속증조.10례위B세포성,3례위T세포성,2례미구분세포류형.유수방자료적B세포성혈관내림파류병6례,균행CHOP방안화료,4례존활,2례사우호흡쇠갈;3례T세포성혈관내림파류병적예후균교차,경CHOP방안화료적효과불가,분별우진단후20 d화3개월사망.결론 이폐부증상화체정위주요표현적혈관내림파류병교위소견,기림상증상무특이성,폐공능검사주요위미산공능장애,상반유혈청유산탈경매승고,흉부CT시쌍폐미만성조색상음영화(혹)다발결절영,부분결절정설형,위우흉막하,상반유폐지기관혈관속증조,종격폐문림파결무종대.경지기관폐활검혹개흉폐활검병리검사가명학진단.
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.