肺淋巴管平滑肌瘤病1例并文献复习

A cases of pulmonary lymphangioleiomyomatosis and literature review

目的提高对罕见病PLAM的认识。方法对我科2011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。结果 PLAM是一种罕见的弥散性肺部疾病,主要发生于育龄期妇女。临床表现为反复发作自发性气胸、活动后呼吸困难、血痰和乳糜胸等。HRCT示两肺弥散分布囊状改变。肺组织活检是确诊的主要依据。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病因不明,预后差,目前无有效的治疗方法 。

Objective To improve the diagnosis and treatment of pulmonary lymphangioleiomyomatosis （PLAM）. Methods One patients with PLAM confirmed by pathological assessment was presented and ana- lyzed along with a review of the related literatures. Results PLAM was a rare pulmonary disease of unknown cause and primarily affects youngwomen. The clinical manifestations were pneumothorax, exertional dyspnea, he- moptysis and chylothorax. HRCT showed bilateral diffuse cystic airspaces change. Lung biopsy is the main evi- dence of the identificationg of the diagnosis. 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 pathogeny of the PLAM currently are unknown and the prognosis was poor. There was no effective treatment at present.