肺淋巴管平滑肌瘤病

Study of pulmonary lymphangioleiomyomatosis

目的 提高对罕见病肺淋巴管平滑肌瘤病 (PL AM)的认识。方法 对我院诊治的患者进行分析 ,并结合文献复习。结果 PL AM是一种罕见的弥漫性肺部疾病 ,临床反复发作自发性气胸或 /和乳糜胸、活动后呼吸困难和痰血等。肺功能呈阻塞性或混合性通气功能障碍 ,低氧血症。胸部 X线表现为两肺弥漫分布网格状阴影 ,高分辨 CT(HRCT)示弥漫分布囊状改变。病理学检查示 :肺组织淋巴管增生和扩张 ,管外平滑肌明显增生。结论 育龄期妇女如反复发生气胸及出现原因不明的呼吸困难、咯血或乳糜胸 ,应疑诊 PL AM,及时行 HRCT及肺功能检查 ,必要时肺活检 ,以明确病理诊断。

Objective To improve the knowledge of pulmonary lymphangioleiomyomatosis (PLAM).Methods The data of the PLAM patient in the study were analyzed and the relevant literature was reviewed.Results PLAM was a rare pulmonary disease of unknown cause. The main clinical manifestations were recurrent pneumothorax or chylothorax, exertional dyspnea and hymoptysis. 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 occurrence, lymphatics dilated and proliferated. Conclusion PLAM should be suspected in women of child bearing age if there were recurrent pneumothorax and unknown etiological dyspnea, hemoptysis or chylothorax. Lung function test and HRCT should be immediately performed for an early diagnosis.