肺淋巴管平滑肌瘤病的临床分析  

Clinical analysis of pulmonary lymphangioleiomyomatosis

作　　者：朴立峰 刘芳[2] 陈中华 

机构地区：[1]辽宁省朝阳市中心医院呼吸科,122000 [2]广州医学院第一附属医院病理科 [3]湖南省岳阳市第一人民医院呼吸科

出　　处：《临床肺科杂志》2006年第3期299-300,共2页Journal of Clinical Pulmonary Medicine

摘　　要：目的探讨肺淋巴管平滑肌瘤病(LAM)的临床特征,治疗和预后。方法回顾性分析1例LAM病人并复习文献78例。结果全部79例患者中,女性占多数74例(93.7%)。发病年龄:69例(87.3%)为育龄期妇女,均经肺组织活检病理确诊。临床表现为呼吸困难(75例),咯血(40例),气胸(38例),乳糜胸(24例),行胸部高分辩CT(HRCT)扫描63例,均显示两肺弥漫性薄壁囊状阴影;行肺功能检查45例,阻塞性通气功能障碍32例,混合性通气功能障碍9例,行血气分析43例,其中低氧血症41例,型呼吸衰竭2例;给予安宫黄体酮及其他药物治疗32例,其中死亡或病情加重19例,其中11例从确诊到死亡时间为0.5-3.5年。结论LAM以呼吸困难,咯血、气胸和乳糜胸为主要临床特征,安宫黄体酮疗效不理想。Objective To study the clinical features, treatment, and prognosis of pulmonary lymphangioleiomyomatosis （LAM）. Methods A retrospective analysis of 79 cases of LAM was made. Among them one case was diagnosed and treated, and the clinical data of the other seventy-eight cases were studied by literature review. Results Among the 79 patients, 74 were female （93.7%） and 5 were male （6.3%）. The onset age ranged between 13 and 69 years old, Sixty-nine patients （87.3%） were women of childbearing age, lung biopsy were made in all the cases. The main clinical manifestations included dysnpea （75 cases ）, hemoptysis （40 cases）, pneumothorax （38 eases）, and chylothorax （24 cases）. The high - resolution computerized tomography conducted in 63 cases showing thin-walled air--dilled cysts throughout both lungs, 45 patients took pulmonary function test with the results of obstructive ventilatory disturbance in 32 cases and mixed ventilatory disturbance in 9 cases. The results of arterial blood gas analysis showed that hypoxemia in 41 eases and respiratory failure （ Ⅰ type） in 2 cases, 32 patients were treated with medroxyprogesterone and the others with drugs. Among them 19 patients＇ conditions got worse or died. 11 cases out of 15 patients died of the disease, and the time between confirmation of diagnosis and death ranged from 0.5 year to 3.5 years. Conclusion The mainclinical manifestations of LAM are dyspnea, hemoptysis, pneumothorax, and chylothorax. Medroxyprogesterone has little curative effect on LAM.

关 键 词：肺淋巴管平滑肌瘤病 肺疾病 

分 类 号：R563.9[医药卫生—呼吸系统]