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Lymphatic Vessels and Lymph Transport

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Lymphatic vessels, known as lymphatics, are crucial in transporting lymph from peripheral tissues to our venous system. This process begins with lymph entering through tiny capillaries that branch through tissues. These capillaries have unique features such as larger diameters, thinner walls, and a distinctive one-way valve system formed by overlapping endothelial cells.
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Updated: May 5, 2026

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

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Published on: May 1, 2015

[Lymphangioleiomyomatosis].

¹Abteilung Pneumologie, Universitätsklinikum Leipzig AöR.

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|November 27, 2013

Summary

This summary is machine-generated.

Lymphangioleiomyomatosis (LAM) is a rare lung disease primarily affecting women. mTORC1 inhibitors offer a promising approach to halt disease progression, improving patient prognosis.

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Area of Science:

Pulmonology
Rare Diseases
Oncology

Background:

Lymphangioleiomyomatosis (LAM) is a rare pulmonary disease affecting predominantly women, with limited data on male patients.
LAM presents in two forms: spontaneous mosaic mutation (S-LAM) and tuberous sclerosis complex-associated LAM (TSC-LAM).
Disease progression is influenced by hormonal factors, with estrogen exacerbating symptoms, and has a variable prognosis despite generally favorable 10-year survival rates.

Purpose of the Study:

To review the clinical presentation, pathophysiology, and therapeutic strategies for Lymphangioleiomyomatosis (LAM).
To highlight the role of the mTORC1 pathway in LAM pathogenesis and the potential of targeted therapies.

Main Methods:

Review of existing literature on LAM.
Analysis of the molecular mechanisms involving mTORC1 pathway activation.
Evaluation of current and emerging therapeutic interventions.

Main Results:

Clinical manifestations include pneumothorax, chylous pleural effusions, and exertional dyspnea, with characteristic cystic changes on HRCT.
Pathophysiology involves mTORC1 pathway hyperactivation, driving protein synthesis, proliferation, and cellular survival.
mTORC1 inhibitors like Sirolimus and Everolimus have shown promise in halting disease progression.

Conclusions:

Targeting the mTORC1 pathway represents a significant therapeutic advance for LAM.
Complementary strategies are under investigation to enhance treatment efficacy.
Lung transplantation remains an option for therapy-resistant LAM cases.