肺淋巴管平滑肌瘤病一例  

Pulmonary lymphangioleiomyomatosis: a case report

作　　者：邹小玲[1] 陈壮桂[2] 冯定云[1] 周宇麒[1] 

机构地区：[1]中山大学附属第三医院呼吸内科,广州510630 [2]中山大学附属第三医院儿科,广州510630

出　　处：《新医学》2016年第6期408-411,共4页Journal of New Medicine

基　　金：国家自然科学基金资助项目(81470219);广东省科技计划项目基金资助项目(2014A020212120)

摘　　要：肺淋巴管平滑肌瘤病(PLAM)是一种罕见的弥漫性肺部疾病,好发于育龄女性,以呼吸困难、咳嗽、咯血及气胸为临床特征,肺功能多为阻塞性通气功能障碍,临床预后差。该文报道1例42岁女性患者,临床表现为气促、呼吸困难及咯血,伴低氧血症及肺弥散功能下降,高分辨率胸部CT显示双肺弥漫性分布薄壁囊腔,经支气管纤维镜病理活组织检查后确诊为PLAM。经口服孕激素治疗8个月后患者临床症状缓解,但肺部病变影像学表现无改善,目前仍在随访观察中,情况尚稳定。Pulmonary lymphangioleiomyomatosis( PLAM) is a rare diffuse pulmonary disease,frequently occurring in women of reproductive age. PLAM is clinically characterized as dyspnea,cough,hemoptysis and pneumothorax. Pulmonary function test reveals obstructive ventilatory disturbance in PLAM patients with poor clinical prognosis. In this article,a 42-year-old woman diagnosed with PLAM was reported. She presented with the clinical manifestations mainly including shortness of breath,dyspnea and hemoptysis,accompanied with hypoxemia and pulmonary diffusion function impairment. High-resolution pulmonary computerized tomography revealed thin-walled air-filled cysts were diffusively distributed throughout bilateral lungs. The diagnosis of PLAM was validated by transbronchoscopy biopsy. Clinical symptoms were alleviated after 8-month of oral administration of progestogen. However,no improvement was obtained in terms of the pulmonary lesions. The patient was being followed up and remained physically stable.

关 键 词：肺淋巴管平滑肌瘤病 呼吸困难 平滑肌增生 

分 类 号：R734.2[医药卫生—肿瘤]