目的 探讨淋巴管肌瘤病（Lymphangioleiomyomatosis，LAM）的临床特点。方法 回顾分析一例肺淋巴管肌瘤病伴 后腹膜淋巴管肌瘤病临床资料，结合文献，分析该病临床特点和诊治现状。结果 本病好发于育龄期妇女，以喘 气、乳糜胸、气胸、咯血等为主要表现，肺功能受损，肺外病变腹膜后常见；肺部CT为大小一致、分布均匀薄壁囊 样改变，可伴液气胸，磨玻璃样改变；腹部CT常为后腹膜囊实性肿块伴淋巴结肿大。结论 当患者具有相应呼吸道 症状，且有典型的肺部及腹部CT表现时，考虑LAM，病检有特异性。

Objective To discuss the clinical features of Lymphangioleiomyomatosis. Methods One case of a pulmonary associated with retroperitoneal lymphangioleiomyomatosis was retrospectively analyzed. Analysis of clinical characteristic and the present situation of diagnosis and treatment of the disease was made combined with the literature. Results The disease mainly occurs in child-bearing women. The main clinical manifestations are pant, chylothorax, pneumothorax, and haemoptysis,etc. Lung function was impaired, retroperitoneal lesions were common extrapulmonary . CT showed thin-walled capsule sample change with same size and uniform distribution, liquid pneumothorax and grinding glass sample change can be associated. Abdominal CT demonstrated retroperitoneal cystic-solid tumors with lymph node enlargement. Conclusion LAM should be considered when patients have corresponding respiratory symptoms, accompanied with typical lung and abdominal CT performance. pathologic examination has specificity.