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Updated: Dec 24, 2025

Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting

Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting

Published on: May 1, 2015

Lymphangioleiomyomatosis.

Kai-Feng Xu^1,2, Wenshuai Xu¹, Song Liu^2,3

¹Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.

Seminars in Respiratory and Critical Care Medicine

|April 13, 2020

Summary

This summary is machine-generated.

Lymphangioleiomyomatosis (LAM) is a rare lung disease affecting women, linked to TSC1/TSC2 mutations. Sirolimus is an approved mTOR inhibitor treatment, with hormone therapies showing preclinical promise.

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Area of Science:

Oncology
Genetics
Pulmonology

Background:

Lymphangioleiomyomatosis (LAM) is a rare, progressive neoplastic disease characterized by lung cysts and kidney angiomyolipomas.
It predominantly affects women and is associated with tuberous sclerosis complex (TSC).
Both LAM and TSC involve TSC1 or TSC2 mutations, leading to mTOR pathway overactivation.

Purpose of the Study:

To review current recommendations for diagnosing and treating LAM.
To highlight recent advancements in understanding and managing this rare disease.

Main Methods:

Literature review of current diagnostic and treatment guidelines for LAM.
Summary of research on the genetic basis and molecular pathways involved in LAM and TSC.
Analysis of therapeutic strategies, including mTOR inhibitors and hormonal therapies.

Main Results:

Sirolimus, an mTOR inhibitor, is an approved treatment for LAM in many countries.
Hormonal therapies have demonstrated preclinical efficacy but require further clinical investigation.
Understanding the genetic links (TSC1/TSC2) and mTOR pathway is crucial for LAM management.

Conclusions:

Current management of LAM involves approved mTOR inhibitors like sirolimus.
Further clinical research is needed for hormone-based therapies in LAM treatment.
LAM diagnosis and treatment benefit from understanding its genetic underpinnings and molecular pathways.