Lymphangioleiomyomatosis and gorham-stout disease: Primary or secondary disorders of the lymphatic system?

M. H. Witte

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Lymphangioleimyomatosis and Gorham-Stout disease, rare disorders which share features of proliferating lymphatic vessels, predominantly in the lung in the former and bone in the latter, commonly manifest as progressive lung disease often associated with life-threatening complications of chylous reflux from central lymphatic obstruction/leakage. This Lymphspiration-Editorial proposes that, rather than a secondary complication, lymphatic obstruction/dysfunction is a much earlier or even the primary event in the pathogenesis of both disorders and that lymphostasis drives the cellular proliferative response in both lung and bone and even distant sites.

Original languageEnglish
Pages (from-to)114-119
Number of pages6
JournalLymphology
Volume50
Issue number3
Publication statusPublished - Sep 2017
Externally publishedYes

Cite this

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title = "Lymphangioleiomyomatosis and gorham-stout disease: Primary or secondary disorders of the lymphatic system?",
abstract = "Lymphangioleimyomatosis and Gorham-Stout disease, rare disorders which share features of proliferating lymphatic vessels, predominantly in the lung in the former and bone in the latter, commonly manifest as progressive lung disease often associated with life-threatening complications of chylous reflux from central lymphatic obstruction/leakage. This Lymphspiration-Editorial proposes that, rather than a secondary complication, lymphatic obstruction/dysfunction is a much earlier or even the primary event in the pathogenesis of both disorders and that lymphostasis drives the cellular proliferative response in both lung and bone and even distant sites.",
keywords = "Benign metastasis, Generalized lymphangiomatosis (GLA), Gorham-Stout disease (GSD), Imaging, Lymphangioleiomyomatosis (LAM), Lymphangioma, Lymphatics, Lymphostasis, Pathogenesis, Treatment",
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Lymphangioleiomyomatosis and gorham-stout disease : Primary or secondary disorders of the lymphatic system? / Witte, M. H.

In: Lymphology, Vol. 50, No. 3, 09.2017, p. 114-119.

Research output: Contribution to journalArticleAcademicpeer-review

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T1 - Lymphangioleiomyomatosis and gorham-stout disease

T2 - Primary or secondary disorders of the lymphatic system?

AU - Witte, M. H.

PY - 2017/9

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N2 - Lymphangioleimyomatosis and Gorham-Stout disease, rare disorders which share features of proliferating lymphatic vessels, predominantly in the lung in the former and bone in the latter, commonly manifest as progressive lung disease often associated with life-threatening complications of chylous reflux from central lymphatic obstruction/leakage. This Lymphspiration-Editorial proposes that, rather than a secondary complication, lymphatic obstruction/dysfunction is a much earlier or even the primary event in the pathogenesis of both disorders and that lymphostasis drives the cellular proliferative response in both lung and bone and even distant sites.

AB - Lymphangioleimyomatosis and Gorham-Stout disease, rare disorders which share features of proliferating lymphatic vessels, predominantly in the lung in the former and bone in the latter, commonly manifest as progressive lung disease often associated with life-threatening complications of chylous reflux from central lymphatic obstruction/leakage. This Lymphspiration-Editorial proposes that, rather than a secondary complication, lymphatic obstruction/dysfunction is a much earlier or even the primary event in the pathogenesis of both disorders and that lymphostasis drives the cellular proliferative response in both lung and bone and even distant sites.

KW - Benign metastasis

KW - Generalized lymphangiomatosis (GLA)

KW - Gorham-Stout disease (GSD)

KW - Imaging

KW - Lymphangioleiomyomatosis (LAM)

KW - Lymphangioma

KW - Lymphatics

KW - Lymphostasis

KW - Pathogenesis

KW - Treatment

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