肺淋巴管平滑肌瘤病

Review of pulmonary lymphangioleiomyomatosis

淋巴管平滑肌瘤病是一种较为罕见的疾病,由于平滑肌样细胞异常增生导致肺囊肿的形成,导致肺功能进行性的下降,最终死亡。本病主要累及妇女,病因目前尚不清楚,亦无特异性治疗,本文对近来关于该病的报道作一综述。

Pulmonary lymphangioleiomyomatosis （LAM） is a rare disease that is characterized by proliferation of abnormal smooth muscle- like cells （LAM cells）, which leads to the formation of lung cysts leading to progressive loss of lung function and ultimately death. It primarily affects women. The etiology of the disease and the efficacy of currently utilized treatments are unknown. The goal is to review the current understanding of LAM.