肺淋巴管平滑肌瘤病九例临床和病理分析

目的 探讨肺淋巴管平滑肌瘤病(pulmonary lymphangionleiomyomatosis,PLAM)的临床和病理特征,提高临床诊治水平.方法 对广州呼吸疾病研究所确诊的9例PLAM患者的临床特点、影像学表现、肺功能及病理学检查进行分析并结合文献复习.结果 9例PLAM患者均为育龄期妇女(20～50岁),临床症状表现为呼吸困难(8/9)、咳嗽(6/9)、咯痰(2/9)、咯血(2/9)、胸痛(3/9)、自发性气胸(6/9)、乳糜胸(3/9)、乳糜性腹腔积液(2/9).胸部X线检查4例表现为双肺弥漫性网状结节影,2例表现为液气胸,2例表现为胸腔积液,1例表现为多发肺小囊状透光区及肺大疱,1例无异常.高分辨率CT具有特征性变化,9例双肺均可见多发小囊状影,直径2～20 mm,壁较薄.2例腹部CT于腹主动脉前可见多个肿大的淋巴结.6例患者进行了肺功能检查,3例通气功能正常,1例呈中度阻塞性通气功能障碍,1例呈重度混合性通气功能障碍,1例呈极重度混合性通气功能障碍,其中1例支气管舒张试验阳性.4例肺弥散功能下降.4例(4/9)经纤维支气管镜肺活检,5例(5/9)经胸腔镜活检,全部经病理确诊.病理学检查显示不同成熟度平滑肌细胞在细支气管壁、肺泡壁、淋巴管壁和血管壁周围增生,肺实质呈囊性变.结论 PLAM表现不典型,易被漏诊、误诊,应提高对本病的认识.

Clinical-pathological anaiysis of nine patients with pulmonary lymphangioleiomyomatosis

Objective To describe the clinical manifestations, diagnosis and treatment of pulmonary lymphangioleiomyomatosis(PLAM). Methods X-ray film, CT-scanning, lung function test, fibro-bronchoscopy and histological examination were performed in all 9 patients. Clinical features were analyzed and compared with reported literatures. Results Syptoms included exertional dyspnea (8/9), cough (6/9),expectoration(2/9), hemoptysis(2/9), chest pain (3/9), pneumothorax ( 6/9 ), chylothorax ( 3/9 ) and chylous ascites(2/9). Radiography showed diffuse reticular shadows in 4 patiens, hydropneumo thorax in 2, pleural effusion in 2 and normal appearance in 1. High resolution chest tomography(HRCT) demonstrated diffused cysts of the lung in all patiens. The cystic air spaces ranged from 2 mm to 20 mm in diameter. Enlarged abdominal lymph nodes were found in 2 patients by abdominal CT-scan. Pulmonary function test showed normal findings in 3 patients,obstructive ventilatory disorder in 1 patient and mixed ventilatory disorderin 1patient. Decreased diffusing capacity for carbon monoxide was found in 4 patients. 4 cases were diagnosed pathologically by biopsy via fiberoptic bronchoseopy and 5 by lung biospy. Pathological features showed abnormal smooth muscle cells(LAM cells) line the airways,lymphatics and blood vessels leading to airflow obstruction and replacement of the lung parenchyma by cysts LAM cells were positive for HMB45.Conclusions As an uncommon disease, PLAM is often misdiagnosed or underdiagnosed. HRCT-scan and fibro-bronchoscopy remain the main method for the diagnosis of PLAM.