肺淋巴管平滑肌瘤1例报道并文献复习

目的提高对罕见病PLAM的认识。方法对我院2006年收治的1例PLAM患者的临床资料进行报道和分析,并复习文献,以提高对该病的认识。结果PLAM是一种罕见的弥散性肺部疾病,临床表现为反复发作自发性气胸、活动后呼吸困难和血痰等。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线表现为两肺弥散分布网格状影,HRCT示两肺弥散分布囊状改变。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示两肺弥散分布囊状改变,临床上应想到PLAM可能。PLAM预后差,目前无有效的治疗方法。

A eases of pulmonary lymphangloleiomyornatosis and literature review

Objective To improve the diagnosis and treatment of pulmonary lymphangioleiomyomatosis(PLAM).Methods One patients with PLAM confirmed by pathological assessment was presented and analyzed along with a review of the related literatures.Results PLAM was 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.Conclusion It should be considered the probability of PLAM when women during children bearing period suffer repeatedly from spontaneous pneumothorax, bloody sputum dyspneic respiration after exercise,and HRCT indicated bilaterai diffuse cystic airspace change;The prognosis of PLAM was poor,and there was no effective treatment at present.