Therapeutic Strategies for Treatment of Pulmonary Lymphangioleiomyomatosis (LAM).

ABSTRACT:

Introduction

Pulmonary lymphangioleiomyomatosis (LAM) is a rare progressive lung disease affecting almost exclusively women. Neoplastic growth of atypical smooth muscle-like cells in the lung induces destruction of lung parenchyma leading to the formation of lung cysts, rupture of which results in spontaneous pneumothorax. LAM occurs sporadically or in association with inherited hamartoma syndrome tuberous sclerosis complex (TSC). Progression of LAM often results in loss of pulmonary function and death. Increasing understanding of neoplastic LAM cell growth is driving the development of therapeutic approaches targeting the disease progression.

Areas covered

This review provides background to understand the rationale for current treatments used in patients with LAM, to critic

SUBMITTER: Krymskaya VP 

PROVIDER: S-EPMC4712719 | biostudies-literature | 2014 Oct

REPOSITORIES: biostudies-literature