围绝经期肺淋巴管平滑肌瘤病1例及临床分析

A Case of Pulmonary Lymphangiomyomatosis in Perimenopausal Women

摘要: 目的：提高对围绝经期妇女肺淋巴管平滑肌瘤病(PLAM)的认识并引起对该病的重视与思考。方法：对我院2013年收治的1例PLAM患者的临床资料进行分析，并复习文献，以提高对本病的认识和诊断水平。结果：PLAM是一种持续发展的弥漫性肺间质疾病，多见于育龄期妇女，但同时也发生于围绝经期患者。结论：对围绝经期妇女发生渐进性呼吸困难不能缓解，类似肺气肿的临床表现及X线胸部阴影，并出现反复气胸或乳糜胸水时同样应考虑为PLAM可能，最好能以肺组织活检明确诊断。该病预后差，目前无有效的预防和治疗方法。

Abstract: Objective: To improve awareness and attention of pulmonary lymphangiomyomatosis (PLAM) in perimenopausal women. Method: A perimenopausal woman suffered from PLAM was analyzed in our department. Result: PLAM is a rare cystic interstitial lung disease that exclusively affects women of child-bearing age. We report a case of perimenopausal female with PLAM. Conclusion: PLAM has been seen in perimenopausal woman and should be considered in patients presenting progressive exertional dyspnea, chronic non-productive cough, spontaneous pneumothorax, chy-lothorax and a plain X-ray chest which shows a hyperinflated lung with reticular or reticulonodular pattern. Lung biopsy should be considered when there is doubt about the diagnosis. The disease prognosis is poor, and currently there are no effective prevention and treatment methods.