淋巴管肌瘤病诊疗进展

Progress in diagnosis and therapy of lymphangioleiomyomatosis

淋巴管肌瘤病是以弥漫性肺囊性病变为特征的一种罕见的缓慢进展的肿瘤性疾病。几乎只发生于女性患者。主要表现为呼吸困难、气胸、乳糜胸。血管内皮生长因子D是其无创且可靠的血清学诊断指标。雷帕霉素抑制剂如西罗莫司可以有效治疗本病。本文介绍最新的诊断和治疗要点。

Lymphangioleiomyomatosis （LAM） is a slowly progressive uncommon neoplasm presented as diffuse cystic lesion in the lung, almost exclusively occurs in women. Dyspnea, pneumothorax, and chylothorax are common presentations in LAM patients. Vascular endothelial growth factor-D in serum is a noninvasive and reliable diagnostic biomarker. Rapamycin inhibitor such as sirolimus is effective for the treatment of LAM. This review discusses current knowledge about diagnosis and therapy of LAM.