[Pulmonary lymphangioleiomyomatosis].
OBJECTIVE
To improve the diagnosis and treatment of pulmonary lymphangioleiomyomatosis (PLAM).


METHODS
Three patients with PLAM confirmed by pathological assessment were presented and relevant literatures were reviewed.


RESULTS
PLAM is a rare pulmonary disease of unknown cause. The clinical manifestations were pneumothorax, exertional dyspnea and hemoptysis. Pulmonary function test showed obstructive or compound ventilative defect and hypoxemia. HRCT showed bilateral diffuse cystic airspaces change. Pathological features showed abnormal smooth muscle proliferation occurred along lymphatics. Lymphatics dilated and proliferated.


CONCLUSIONS
The prognosis of PLAM is poor. There is no effective method for the treatment of this disease at present.
Jin
M
M
Pulmonary Department, Zhongshan Hospital, Shanghai Medical University, Shanghai 200032, China.
Cai
Y
Y
Zhang
Z
Z
D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000473:pathology*; D018192:Lymphangioleiomyomatosis / Q000473:pathology*; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000473:pathology