PDF(Pubmed)
Abstract：
Lymphangioleiomyomatosis (LAM) is an abnormal proliferation of smooth muscle-like cells and may occur sporadically or in association with tuberous sclerosis complex. Patients are typically female, nonsmoking and may have cystic lung disease with pneumothorax. Diagnosis can be made by compatible imaging findings with a history of tuberous sclerosis complex, or in conjunction with vascular endothelial growth factor-D 800 pg/ml or greater, a highly specific finding. Sirolimus is first line treatment for LAM.
摘要：
淋巴管平滑肌瘤病（LAM）是平滑肌样细胞的异常增殖，可能偶尔发生或与结节性硬化症有关。患者通常是女性，不吸烟，可能有囊性肺疾病伴气胸。诊断可以通过符合结节性硬化症病史的影像学发现，或与血管内皮生长因子-D结合800pg/ml或更高，一个非常具体的发现。西罗莫司是LAM的一线治疗。
