淋巴管平滑肌瘤病

Lymphangioleiomyomatosis.

作者信息

Glassberg Marilyn K

机构信息

Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Vascular Biology Institute, University of Miami School of Medicine, 1600 North West 10th Avenue, Miami, FL 33136, USA.

出版信息

Clin Chest Med. 2004 Sep;25(3):573-82, vii. doi: 10.1016/j.ccm.2004.05.003.

DOI:10.1016/j.ccm.2004.05.003

PMID:15331193

Abstract

Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease predominately affecting women. The disease is characterized by peribronchial, perivascular, and perilymphatic proliferation of smooth muscle like cells resulting in vascular and airway obstruction and cyst formation. The natural history of pulmonary LAM is unknown, and it displays remarkable diversity in its clinical course. More than 400 citations are currently listed in the literature for LAM. This article reviews and updates the rapidly expanding knowledge about LAM.

摘要

淋巴管平滑肌瘤病（LAM）是一种罕见的特发性疾病，主要影响女性。该病的特征是支气管周围、血管周围和淋巴管周围平滑肌样细胞增殖，导致血管和气道阻塞以及囊肿形成。肺LAM的自然病程尚不清楚，其临床过程表现出显著的多样性。目前文献中列出了400多篇关于LAM的引用。本文回顾并更新了关于LAM迅速扩展的知识。