肺淋巴管肌瘤病11例报告并文献复习

目的 探讨肺淋巴管肌瘤病( PLAM)的临床和预后.方法 分析经治的11例PLAM病人的资料,并复习文献.结果 11例均为女性；年龄31 ～55岁,平均(44±9)岁；均经肺组织活检明确病理；主要临床表现为进行性呼吸困难10例(发生率91％),咳嗽6例(55％),咯血2例(18％),乳糜胸3例(27％),气胸3例(27％)等；11例患者均进行肺功能检测,均有弥散功能下降,9例行动脉血气分析,其中7例表现为低氧血症,4例合并呼吸衰竭；11例行胸部高分辨率CT (HRCT),均显示两肺弥漫性薄壁囊状阴影.结论 PLAM以呼吸困难,咳嗽,气胸和乳糜胸为主要临床表现；双肺弥漫性薄壁囊状阴影为影像学特征表现；肺组织活检是确诊的主要依据.

Clinical analysis of pulmonary lymphangioleiomyomatosis

Objective To study the clinical features,treatment,and prognosis of pulmonary lymphangioleiomyomatosis(PLAM).Methods Retrospective analyzed the clinical materials of 11cases of the PLAM and review the documents.Results 11 cases were femail;the age of onset ranged 31~55 years,with an average age(44±9) years.All of the cases were diagnosed by the pathological examination of lung biopsy.The main clinical manifestations included dyspea(10 cases,91%),cough(6 cases,55%),hemoptysis(2 cases,18%),pneumothorax(3 cases,27%),and chylothorax(3 cases,27%).11 cases took pulmonary function test with the result of reduction of carbon dioxide duffusing capacity in all cases.Arterial blood gas analysis was made among 9 patients and showed hypoxemia in 7 cases and respiratory failure in 4 cases.High resolution computerized tomography conducted in 11 cases showed diffused thin-walled air-filled cysts throughout both lungs.Six out of 11 patients were misdiagnosed as other diseases at the first visit.The time between onset of symptoms and confirmation of diagnosis ranged 0.5~18 years.Four patients of 11 cases were treated with injection of the medroxyprogesterone,three cases with oral medroxyprogesterone,two cases took bronchodilators treatment,one case took family oxygen.Six patients died of respiratory failure,pneumothorax and the lung infection.Conclusion The main clinical manifestations of PLAM are dyspea,cough,pneumothorax and chylothorax;Lung biopsy is the main evidence of the identification of the diagnosis.Medroxyprogesterone is of little curative effect on PLAM,recently reseachments showed inhibitors of mTOR may the future choice of treatments of PLAM.