Johnson S F, Davey D D, Cibull M L, Schwartz R W, Strodel W E
Department of Surgery, Chandler Medical Center, University of Kentucky, Lexington 40536-0084.
Am Surg. 1993 Jul;59(7):395-9.
Lymphangiomyomatosis (LAM) is a progressive disease afflicting women of childbearing age. LAM remains a rare disease, unfamiliar to many clinicians. It usually poses a diagnostic dilemma for the primary physician, possibly resulting in a delayed or missed diagnosis, unnecessary operative intervention, and inappropriate therapy. LAM is characterized microscopically by abnormal smooth muscle proliferation causing gradual obstruction of small airways, lymphatics, and vasculature. The proliferation eventually results in a characteristic clinical syndrome of progressive dyspnea, recurrent pneumothorax, chylous effusion, and hemoptysis. Despite a variety of treatment regimens developed since the first description of LAM, patient survival has not improved appreciably. Most patients die within 10 years of the time of diagnosis. This report presents a patient with LAM and a review of the literature.
淋巴管平滑肌瘤病（LAM）是一种累及育龄女性的进行性疾病。LAM仍然是一种罕见疾病，许多临床医生对此并不熟悉。它通常给初级医生带来诊断难题，可能导致诊断延迟或漏诊、不必要的手术干预及不恰当的治疗。LAM在显微镜下的特征是异常平滑肌增生，导致小气道、淋巴管和脉管系统逐渐阻塞。这种增生最终导致出现进行性呼吸困难、复发性气胸、乳糜胸和咯血的典型临床综合征。尽管自首次描述LAM以来已制定了多种治疗方案，但患者生存率并未显著提高。大多数患者在诊断后10年内死亡。本报告介绍了一名LAM患者并对文献进行了综述。
