肺淋巴管平滑肌瘤病临床分析

Clinical analysis of pulmonary lymphangioleiomyomatosis.

目的探讨肺淋巴管平滑肌瘤病的临床特征，提高对肺淋巴管平滑肌瘤病的认识。方法对2例肺淋巴管平滑肌瘤病患者的临床资料进行分析，并结合文献进行复习。结果肺淋巴管平滑肌瘤病临床表现为呼吸困难，气胸，乳糜胸，肺功能呈阻塞性或限制性通气功能障碍，弥散功能障碍。高分辨CT示两肺广泛弥漫性分布的薄壁小囊状病变。病理特点为肺淋巴管、小血管、小气道的管壁平滑肌细胞弥漫增生。结论育龄期妇女如反复出现气促或乳糜胸，胸部CT示两肺弥漫性囊状改变，即应考虑肺淋巴管平滑肌瘤病可能。肺淋巴管平滑肌瘤病预后差，目前无有效治疗方法。

Objective To investigate clinical features of pulmonary lymphangioleiomyomatosis （PLAM）, and to improve the diagnosis and treatment. Methods Two patients with PLAM confirmed by pathological assessment were presented and the relevant literatures were reviewed. Results The disease was characterized by progressive pulmonary cystic change, recurrent pneumothorax, chylous pleural collections and,in most cases,progressive respiratory failure. The most frequent pulmonary function abnormalities in PLAM were airflow obstruction and a decreased lung diffusion capacity. The principal pulmonary roentgenographic finding in patients with PLAM was the presence of cysts on lung computed tomography （CT） scans that were well circumscribed, round or oval, and thinwalled, scattered throughout the lungs. Lymphangioleiomyomas diagnosis was made by a combination of clinical features and computed tomography scanning. Conclusion No effective treatment currently exists for this progressive disorder. The diagnosis of PLAM should be considered in women present recurrent pneumothorax, chylous pleural effusions, or an unexplained decrease in exercise tolerance.