肺淋巴管肌瘤病的临床分析

目的探讨肺淋巴管肌瘤病 (LAM)的临床特征、疗效和预后,提高对该病的认识. 方法回顾性分析经治的2例LAM病人并复习文献14例LAM病人的临床资料. 结果经治的2例病人均以呼吸困难为主要症状,肺功能检查均为阻塞性通气功能障碍和弥散功能减低.16例LAM病人中女性15例(94%),男性1例(6%);发病年龄5～69(35±15)岁,13例(81%)为中青年生育期女性.均经肺组织活检病理确诊.主要临床表现为呼吸困难(15例)、咯血(10例)、气胸(7例)、乳糜胸(7例)等.14例行胸部高分辨CT(HRCT)扫描,均显示两肺弥漫性薄壁囊状阴影.7例行肺功能检查,均有弥散功能减低,5例有肺阻塞性通气功能障碍,2例有混合性通气功能障碍.11例作了动脉血气分析,其中8例表现为低氧血症,5例合并呼吸衰竭.16例病人首次就诊时均被误诊为其他疾病,自首发症状至确诊LAM经历2～276个月,中位数为28个月.6例病人应用安宫黄体酮治疗6～50个月,2例病情稳定,2例病情加重,2例死亡.16例中6例(38%)在症状出现后1.5～26年内因呼吸衰竭、气胸和肺部感染死亡. 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.探讨病因及寻找有效的疗法是今后研究的重要课题.

Clinical analysis of pulmonary lymphangioleiomyomatosis

OBJECTIVE: To study the clinical features, treatment, and prognosis of pulmonary lymphangioleiomyomatosis (LAM). METHODS: A retrospective analysis of 16 cases of LAM was made, among which two were diagnosed and treated in the Chinese PLA General Hospital, and the clinical data of the other fourteen cases were studied by literature review. RESULTS: Among the 16 patients, 15 were female (94%) and 1 was male (6%). The age of onset ranged 5 - 69 years, with an average age of onset of 35 +/- 15 years. Thirteen patients (81%) were women of childbearing age. The main clinical menifestations included dyspnea (15 cases, 94%), hemoptysis (10 cases, 62%), pneumothorax (7 cases, 44%), and chylothorax (7 cases, 44%). High-resolution computerized tomography conducted in 14 cases showed thin-walled air-filled cysts throughout both lungs. Seven patients took pulmonary function test with the results of obstructive ventilatory disturbance in 5 cases, mixed ventilatory disturbance in 2 cases, and reduction of carbon dioxide duffusing capacity in all cases. Arterial blood gas analysis was made among 11 patients and showed hypoxemia (blood oxygen < 80 mm Hg) in 8 cases and respiratory failure in 5 cases. All of the patients were misdiagnosed as other diseases at the first visit. The time between onset of symptoms and confirmation of diagnosis ranged 2 - 276 months (median 28 months). Six patients were treated with medroxyprogesterone for 6-50 months. Among them two patient' condition remained stable, two patients' condition was worsened, and two died. Six out of the 16 patients (38%) died of respiratory failure, pneumothorax, or lung infection 1.5 to 26 years after the onset. CONCLUSION: The main clinical manifestations of LAM are dyspnea, hemoptysis, pneumothorax, and chylothorax. Medroxyprogesterone is of little curative effect on LAM. The main task in LAM research is to find out its etiology and effective treatment.