肺淋巴管平滑肌瘤病2例诊疗分析

目的：提高对肺淋巴管平滑肌瘤病（PLAM）的认识,减少误诊。方法：对2例PLAM的临床资料并结合文献进行分析。结果：PLAM为一种弥漫性肺间质疾病,临床表现呼吸困难、咯血、气胸、乳糜胸等,极易被误诊。胸部HRCT具有特征性改变,显示两侧对称弥漫性广泛囊状影。结论：对于年轻育龄期女性反复出现气胸、乳糜胸,胸部HRCT提示囊状改变,需考虑PLAM,预后差,无特效治疗。

Diagnosis Analysis on 2 Cases of Pulmonary Lymphangioleiomyomatosis

Objective: Improving the cognition on pulmonary lymphangioleiomyomatosis and reduce the misdiagnosis. Methods: Making an analysis on the clinical data as well as documents of the two pulmonary lymphangioleiomyomatosis. Results: Pulmonary lymphangioleiomyomatosis is a diffuse pulmonary interstitial disease. Its clinical symptoms are anhelation, hemoptysis, pneumothorax, chylothorax, etc. and could be misdiagnosed very easily. The thorax HRCT shows a characteristic change with symmetric diffuse broad cystic shadow on both sides. Conclusion: If pneumothorax, chylothorax occur repeatedly to young females in child-bearing period and thorax HRCT shows a cystic change, pulmonary lymphangioleiomyomatosis should be taken into consideration, which is of poor prognosis, no specific treatment.