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Updated: Mar 16, 2026

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.

Simon R Johnson¹, Angelo M Taveira-DaSilva², Joel Moss³

¹Division of Respiratory Medicine, National Centre for Lymphangioleiomyomatosis, University Hospitals NHS Trust, University of Nottingham, Nottingham, NG7 2UH, UK.

Clinics in Chest Medicine

|August 13, 2016

Summary

This summary is machine-generated.

Lymphangioleiomyomatosis (LAM) is a rare lung disease causing cysts and respiratory failure. Treatment involves supportive care, bronchodilators, and mTOR inhibitors for progressive cases.

Keywords:

Angiomyolipoma Cystic lung disease Lymphatic disease Pneumothorax mTOR inhibitor

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Area of Science:

Pulmonary Medicine
Rare Diseases
Genetics

Background:

Lymphangioleiomyomatosis (LAM) is a rare, multisystem disorder primarily affecting women.
It can manifest sporadically or in association with tuberous sclerosis complex.
The disease is characterized by the proliferation of abnormal smooth muscle cells in the lungs, leading to cyst formation and destruction of lung parenchyma.

Purpose of the Study:

To summarize the key aspects of Lymphangioleiomyomatosis.
To outline the clinical presentation, associated conditions, and current treatment strategies for LAM.

Main Methods:

Review of existing literature on Lymphangioleiomyomatosis.
Synthesis of information regarding pathophysiology, clinical manifestations, and therapeutic interventions.

Main Results:

LAM causes progressive lung cysts, dyspnea, recurrent pneumothorax, and potential respiratory failure.
Extrapulmonary manifestations include lymphatic disease and renal angiomyolipomas.
Treatment options range from supportive care and bronchodilators to mTOR inhibitors for advanced disease.

Conclusions:

Lymphangioleiomyomatosis is a complex disease requiring a multidisciplinary approach.
Early diagnosis and management are crucial for improving patient outcomes.
Targeted therapies like mTOR inhibitors offer new hope for patients with progressive LAM.