淋巴管平滑肌瘤病(lymphangioleiomyomatosis,LAM)是一种主要影响育龄期女性的罕见疾病。该病为多系统疾病,主要累及肺脏,表现为两肺弥漫性囊泡改变,常合并肾血管平滑肌脂肪瘤(angiomyolipoma,AML)、后腹膜、盆腔淋巴结肿大等肺外表现。临床上主要表现为进行性加重的呼吸困难、反复自发性气胸、顽固性乳糜胸等。该病的发生 Lymphangioleiomyomatosis (LAM) is a rare disease that affects mainly females of childbearing age. The disease is a multi-system disease, mainly involving the lungs, manifested as diffuse vesicular changes in both lungs, often associated with renal angiomyolipoma (angiomyolipoma, AML), retroperitoneal, pelvic lymph nodes and other extrapulmonary manifestations. Clinically manifested as progressive dyspnea, recurrent spontaneous pneumothorax, intractable chylothorax and so on. The occurrence of the disease