肺淋巴管肌瘤病9例临床分析

Pulmonary lymphangioleiomyomatosis:a report of 9 cases

目的探讨肺淋巴管肌瘤(PLAM)的临床表现及诊治方法。方法报道并临床分析2005—2008年中南大学湘雅二院收治的具有较完整资料的9例肺淋巴管肌瘤病例,结合文献进行复习。结果 9例均为女性。均有活动后气促,1例有乳糜腹,2例有乳糜胸,3例有气胸,6例有咯血。9例高分辨CT(HRCT)均示肺部弥漫分布薄壁囊腔透光区的PLAM特征性表现而临床诊断。4例行病理组织检查确诊。国内首次报道予雷帕霉素治疗LAM且疗效满意的病例。结论 HRCT检查对PLAM具诊断价值,对育龄妇女发生的渐进性呼吸困难、气胸、乳糜胸、咯血应及时进行胸部HRCT检查,雷帕霉素给此病的治疗带来新希望。

Objective To discuss the clinical features, diagnosis, management of pulmonary lymphangioleiomyomatosis （ PLAM ）. Methods The clinical data of 9 patients with PLAM, Who were treated in our hospital over the past 3 years, were retrospective analyzed. Results All the 9 cases were women and all had dyspnea after activity. One had Chylous Ascites, two had Chylothorax, three had pneumothorax and six had hemoptysis. The diagno- sis of PLAM was made according to high-resolution CT （HRCT） which showed multiple small thin-wall cysts dis- tributing in the bilateral lung. PLAM was confirmed by biopsy in 4 cases. Here we had the first reported case of PLAM in China with the clinical features improved after the treatment with rapamyein. Conclusion HRCT can confirm diagnosis of PLAM. HRCT should be performed in time for women of child-bearing age with progressive dyspnea, panting, haemoptysis, or chylous pleural effusion. Rapamycin may be a promising drug for PLAM therapy.