Ussavarungsi Kamonpun, Laroia Archana T, Burger Charles D
Division of Pulmonary and Critical Care, Department of Internal Medicine, University of Iowa, Iowa City, IA, USA.
Division of Cardiovascular and Pulmonary Imaging, Department of Radiology, University of Iowa, Iowa City, IA, USA.
Lung India. 2019 Jul-Aug;36(4):349-352. doi: 10.4103/lungindia.lungindia_433_18.
Lymphangioleiomyomatosis (LAM) is a rare disease associated with cystic lung destruction and abdominal tumors, including lymphangioleiomyomas, which frequently occur in the retroperitoneal region. Sirolimus therapy is currently recommended for LAM patients with abnormal or declining lung function with an adjusted dose to maintain a serum trough level of 5-15 ng/mL. We describe a significant reduction of retroperitoneal lymphangioleiomyomas after treatment with low-dose sirolimus therapy (serum trough level <5 ng/mL) in a patient with sporadic LAM.
淋巴管平滑肌瘤病（LAM）是一种罕见疾病，与肺囊性破坏和腹部肿瘤有关，包括淋巴管平滑肌瘤，后者常发生于腹膜后区域。目前推荐西罗莫司疗法用于肺功能异常或下降的LAM患者，需调整剂量以维持血清谷浓度在5-15 ng/mL。我们描述了1例散发性LAM患者在接受低剂量西罗莫司治疗（血清谷浓度<5 ng/mL）后腹膜后淋巴管平滑肌瘤显著缩小的情况。
