淋巴血管平滑肌增生症──病例報告

Lymphangioleiomyomatosis – A Case Report

淋巴血管平滑肌增生症好發於生育年齡女性，造成病人產生漸進性阻塞性肺病、氣胸及肋膜積水甚至是呼吸衰竭。喘是病人最主要的表現，此外雖然在胸部X光及電腦斷層上這個疾病都有較特別的表現但診斷方面主要還是要仰賴病理切片，至於治療方面目前並沒有較好的方法。我們報告這一罕見病例是一位40歲的女性外勞，她主要是因為喘及胸痛來到我們胸腔科門診，經由陸續的檢查，包含胸部x光片、電腦斷層以及胸腔鏡切片而獲得確定的診斷。因此我們提出來和大家分享經驗並對於這個疾病做介紹。

Lymphangioleiomyomatosis (LAM) is an uncommon disease which affects women during their productive years. It can involve the bronchioles, blood vessels and lymphatic system of the lungs and cause obstructive lung disease, pneumothorax and pleural effusion. Dyspnea is the most common presenting symptom. Although there are specific features found on chest X-ray and computerized tomography (CT) scan, the definitive diagnosis is made by biopsy. No standard treatment is currently available. We present a 40 year-old female who visited our chest outpatient department due to dyspnea and chest pain. After a series of examinations including chest X-ray, CT scan and thoracoscopic biopsy, the diagnosis of LAM was made. We describe this unusual case and review the literature about the incidence, symptoms, diagnosis, and treatment of LAM.