肺淋巴管肌瘤病(PLAM)是一种罕见的以肺淋巴管旁平滑肌细胞广泛不典型增生为主的弥漫性肺部疾病,好发于育龄期女性,可累及淋巴管、肾脏及全身其他器官[1]。临床上以进行性呼吸困难、咳血、反复气胸为主要表现。胸部CT检查可表现一定的特征,而同时行腹盆部CT检查发现肺外病变则有助于早期诊断。1资料与方法1.1临床资料搜集2007年1月~2014年12月在 Pulmonary lymphangiomatosis (PLAM) is a rare diffuse pulmonary disease characterized by extensive atypical hyperplasia of pulmonary lymphatic para-smooth muscle cells. It occurs predominantly in women of childbearing age and may involve lymphatic vessels, kidneys and other general Organ [1]. Clinically, progressive dyspnea, hemoptysis, repeated pneumothorax as the main performance. Chest CT examination can show some characteristics, while at the same time abdominal part of the CT examination found that extrapulmonary lesions are helpful to early diagnosis. 1 Materials and Methods 1.1 clinical data collected in January 2007 ~ December 2014 at