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Updated: May 14, 2026
07:36
Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting
Published on: May 1, 2015
Lymphangioleiomyomatosis: current and future
Maria Mavroudi1, Paul Zarogoulidis, Nikolaos Katsikogiannis
1Pulmonary Department, "G. Papanikolaou" General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece;
Journal of Thoracic Disease
|February 2, 2013
Summary
Lymphangioleiomyomatosis (LAM) is a rare lung disease primarily affecting women, characterized by abnormal cell growth and cystic lung changes. Research into its genetic pathways may pave the way for future targeted therapies.
Area of Science:
- Pulmonology
- Oncology
- Genetics
Background:
- Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease predominantly affecting women of reproductive age.
- It can occur sporadically or in association with Tuberous Sclerosis Complex.
- LAM involves cystic lung remodeling due to abnormal smooth muscle-like cell proliferation and extrapulmonary manifestations.
Purpose of the Study:
- To summarize the key characteristics, clinical manifestations, and current therapeutic landscape of Lymphangioleiomyomatosis.
- To highlight the potential for targeted therapies based on ongoing research into disease pathogenesis.
Main Methods:
- Review of existing literature on Lymphangioleiomyomatosis.
- Analysis of genetic and molecular pathways implicated in LAM pathogenesis.
Main Results:
- LAM presents with progressive dyspnea, pneumothorax, and chylous effusions.
- Extrapulmonary signs include lymphadenopathy and angiomyolipomas.
- Current treatments are not curative, but research is advancing.
Conclusions:
- Understanding the genetic and molecular basis of LAM is crucial for developing effective treatments.
- Targeted therapies hold promise for managing this rare lung disease.
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