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目的探讨肺淋巴管肌瘤(PLAM)的临床表现及诊治方法。方法报道并临床分析2005—2008年中南大学湘雅二院收治的具有较完整资料的9例肺淋巴管肌瘤病例,结合文献进行复习。结果 9例均为女性。均有活动后气促,1例有乳糜腹,2例有乳糜胸,3例有气胸,6例有咯血。9例高分辨CT(HRCT)均示肺部弥漫分布薄壁囊腔透光区的PLAM特征性表现而临床诊断。4例行病理组织检查确诊。国内首次报道予雷帕霉素治疗LAM且疗效满意的病例。结论 HRCT检查对PLAM具诊断价值,对育龄妇女发生的渐进性呼吸困难、气胸、乳糜胸、咯血应及时进行胸部HRCT检查,雷帕霉素给此病的治疗带来新希望。
Objective To investigate the clinical manifestations and diagnosis and treatment of lung lymphangioma (PLAM). Methods Nine cases of lung lymphangiomyoma with complete data were reported and analyzed clinically from 2005 to 2008 in Xiangya Hospital of Central South University and reviewed in the literature. Results 9 cases were female. All had episodes of shortness of breath. One had chylous abdomen, two had chylothorax, three had pneumothorax, and six had hemoptysis. 9 cases of high resolution CT (HRCT) showed diffuse distribution of the lung parenchyma luminal PLAM characteristics of the performance of clinical diagnosis. 4 routine pathological examination confirmed. For the first time reported to rapamycin treatment of LAM and the effect of satisfactory cases. Conclusions HRCT has diagnostic value for PLAM, progressive dyspnea in women of childbearing age, pneumothorax, chylothorax, hemoptysis should be chest CT HRCT, rapamycin brings new hope for the treatment of this disease.