RESUMEN
Objective To improve the diagnosis and treatment of pulmonary lymphangiomyomatosis (PLAM). Methods The clinical data of 1 PLAM case in our hospital was analysed, along with a review of the related literatures.Results PLAM was a rare diffuse interstitial plumonary disease of unknown cause, which was oiraracterized by recurrent spontaneous pneumothorax shortness of breath (dyspnea) with physical activity, haemopthsis and chylothorax. Radiological appearances were extensive bilateral linear pattern, and honeycomb pattern in the end. High resolution CT scan (HRCT) showed multiple thin walled cysts distributed evenly throughout the bilateral lung fields with normal intervening lung parenchyma. Pathological characters were abnormal, pervasice multiplication of smooth muscle of cell in and around the small airway, lymphatic and small blood vessels. Immunohistochemical test showed HMB45(+).Conclusion It should be considered the probability of PLAM when women of childbearing ages have some clinical manifestations as follows, the increasing dyspnoea that can not be relieved similar to emphysema, high- resolution CT scan (HRCT) shows multiple thin walled cysts distributed evenly throughout the bilateral lung fields with normal intervening lung parenchyma, and recurrent pneumothorax or chylothorax. The optimal confirm diagnostic method is lung tissue biopsy.