淋巴管肌瘤病的研究新进展  

The latest research progress of lymphangiomyomatosis

作　　者：杨晟楠 赵勇[1] 李月川[1] 贾玮[1] YANG Sheng-nan;ZHAO Yong;LI Yue-chuan;JIA Wei(Department of Respiratory and Critical Care Medicine,Tianjin Chest Hospital,Tianjin 300222,China)

机构地区：[1]天津市胸科医院呼吸与危重症医学科,300222

出　　处：《天津医药》2020年第8期790-795,共6页Tianjin Medical Journal

摘　　要：淋巴管肌瘤病(LAM)是一种罕见的全身性疾病,主要累及育龄期女性。LAM通常具有侵袭性,最终会导致呼吸衰竭。该疾病通过淋巴管扩散,肺脏是主要的靶器官,可导致肺脏的囊性重塑。近年来的研究增强了对LAM发病机制的了解,除哺乳动物雷帕霉素靶蛋白(mTOR)通路过度激活外,NR2F2突变也可能是其发病机制。一些影像学新技术的研发,可能为评价疗效和随访提供更加安全的手段。尽管mTOR抑制剂雷帕霉素在LAM患者中显示出某些益处,可以稳定肺功能和改善生活质量,但停止治疗后疾病仍继续进展,而免疫疗法可能为LAM提供新的治疗方向。本综述将重点介绍LAM发病机制和治疗方面的新进展。Lymphangioleiomyomatosis(LAM)is a rare systemic disease mainly affecting reproductive women.LAM is generally aggressive in nature and ultimately results in respiratory failure.The disease spreads through lymphatic vessels,and the lung is the main target organ,leading to cystic remodeling of the lung.In the last few years,studies have enhanced the understanding of LAM pathogenesis.In addition to the over activation of mTOR pathway,NR2F2 mutation is also a possible pathogenesis.The research and development of some new imaging technologies may provide more safe means for evaluation of efficacy and follow-up.Although rapamycin,the mTOR inhibitor,has shown some benefit in LAM patients,which can stabilize lung function and improve quality of life,the disease continues to progress after the cessation of treatment.Immunotherapy may provide a new treatment direction for LAM.This review will focus on the new progress in pathogenesis and treatment of LAM.

关 键 词：淋巴管肌瘤病 发病机制 病理学 体层摄影术 X线计算机 

分 类 号：R563.9[医药卫生—呼吸系统]