肺淋巴管平滑肌瘤病

Pulmonary lymphangioleiomyomatosis

目的 提高对罕见病肺淋巴管平滑肌瘤病 (PLAM)的认识。方法 对 1992年以来诊治的 3例PLAM患者的临床资料进行分析 ,并结合文献复习。结果 PLAM是一种罕见的弥漫性肺部疾病 ,临床表现为反复发作自发性气胸、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 ,动脉血气示低氧血症。胸部X线表现为两肺弥漫分布网格状影 ,高分辨CT(HRCT)示两肺弥漫分布囊状改变。病理学检查示肺组织淋巴管增生和扩张 ,管外平滑肌细胞明显增生。结论 育龄期妇女 ,如反复出现自发性气胸、痰血、活动后呼吸困难 ,胸部HRCT示两肺弥漫分布囊状改变 ,临床上应想到PLAM可能。PLAM预后差 。

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.