Lymphangioleiomyomatosis: Current understanding and potential treatments.
Identifieur interne : 000D65 ( Main/Exploration ); précédent : 000D64; suivant : 000D66Lymphangioleiomyomatosis: Current understanding and potential treatments.
Auteurs : Lyn M. Moir [Australie]Source :
- Pharmacology & therapeutics [ 1879-016X ] ; 2016.
Descripteurs français
- KwdFr :
- Animaux, Antinéoplasiques (pharmacologie), Antinéoplasiques (usage thérapeutique), Humains, Lymphangioléiomyomatose (anatomopathologie), Lymphangioléiomyomatose (génétique), Lymphangioléiomyomatose (métabolisme), Lymphangioléiomyomatose (traitement médicamenteux), Mutation (génétique), Poumon (), Poumon (anatomopathologie), Qualité de vie, Sirolimus (pharmacologie), Sirolimus (usage thérapeutique), Sérine-thréonine kinases TOR (antagonistes et inhibiteurs).
- MESH :
- anatomopathologie : Lymphangioléiomyomatose, Poumon.
- antagonistes et inhibiteurs : Sérine-thréonine kinases TOR.
- génétique : Lymphangioléiomyomatose, Mutation.
- métabolisme : Lymphangioléiomyomatose.
- pharmacologie : Antinéoplasiques, Sirolimus.
- traitement médicamenteux : Lymphangioléiomyomatose.
- usage thérapeutique : Antinéoplasiques, Sirolimus.
- Animaux, Humains, Poumon, Qualité de vie.
English descriptors
- KwdEn :
- Animals, Antineoplastic Agents (pharmacology), Antineoplastic Agents (therapeutic use), Humans, Lung (drug effects), Lung (pathology), Lymphangioleiomyomatosis (drug therapy), Lymphangioleiomyomatosis (genetics), Lymphangioleiomyomatosis (metabolism), Lymphangioleiomyomatosis (pathology), Mutation (genetics), Quality of Life, Sirolimus (pharmacology), Sirolimus (therapeutic use), TOR Serine-Threonine Kinases (antagonists & inhibitors).
- MESH :
- chemical , antagonists & inhibitors : TOR Serine-Threonine Kinases.
- chemical , pharmacology : Antineoplastic Agents, Sirolimus.
- chemical , therapeutic use : Antineoplastic Agents, Sirolimus.
- drug effects : Lung.
- drug therapy : Lymphangioleiomyomatosis.
- genetics : Lymphangioleiomyomatosis, Mutation.
- metabolism : Lymphangioleiomyomatosis.
- pathology : Lung, Lymphangioleiomyomatosis.
- Animals, Humans, Quality of Life.
Abstract
Lymphangioleiomyomatosis (LAM) is a rare neoplastic disease affecting predominantly young women. Clinical symptoms of this progressive disease include dyspnoea, cough, recurrent pneumothorax, hemoptysis and chylothorax. LAM is generally aggressive in nature and ultimately results in respiratory failure. Important hallmark features of this metastatic disease include the formation of lesions of abnormal smooth muscle cells, cystic destruction of the lung tissue and lymphangiogenesis affecting the lungs, abdomen and lymphatics. Research over the last 10-15 years has significantly enhanced our understanding of the molecular and cellular processes associated with LAM. These processes include mutational inactivation of the tuberous sclerosis complex genes, TSC1 and TSC2, activation of the mammalian target of rapamycin (mTOR) pathway, enhanced cell proliferation and migration, lymphangiogenesis, metastatic spread through the blood and lymphatic circulations, sex steroid sensitivity and dysregulated autophagy. Despite this increased knowledge there is currently no cure for LAM and treatment options remain limited. Whilst the mTOR inhibitor rapamycin has shown some benefit in patients with LAM, with stabilisation of lung function and improved quality of life, cessation of treatment results in recurrence of the disease progression. This highlights the urgent need to identify novel targets and new treatment regimens. The focus of this review is to summarise our current understanding of the cellular and molecular processes associated with LAM and highlight emerging treatments.
DOI: 10.1016/j.pharmthera.2015.12.008
PubMed: 26713679
Affiliations:
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Moir</name><affiliation wicri:level="4"><nlm:affiliation>Woolcock Institute of Medical Research, The University of Sydney, Sydney, NSW, Australia; Discipline of Pharmacology, Sydney Medical School, The University of Sydney, Sydney, NSW, Australia. 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Clinical symptoms of this progressive disease include dyspnoea, cough, recurrent pneumothorax, hemoptysis and chylothorax. LAM is generally aggressive in nature and ultimately results in respiratory failure. Important hallmark features of this metastatic disease include the formation of lesions of abnormal smooth muscle cells, cystic destruction of the lung tissue and lymphangiogenesis affecting the lungs, abdomen and lymphatics. Research over the last 10-15 years has significantly enhanced our understanding of the molecular and cellular processes associated with LAM. These processes include mutational inactivation of the tuberous sclerosis complex genes, TSC1 and TSC2, activation of the mammalian target of rapamycin (mTOR) pathway, enhanced cell proliferation and migration, lymphangiogenesis, metastatic spread through the blood and lymphatic circulations, sex steroid sensitivity and dysregulated autophagy. Despite this increased knowledge there is currently no cure for LAM and treatment options remain limited. Whilst the mTOR inhibitor rapamycin has shown some benefit in patients with LAM, with stabilisation of lung function and improved quality of life, cessation of treatment results in recurrence of the disease progression. This highlights the urgent need to identify novel targets and new treatment regimens. The focus of this review is to summarise our current understanding of the cellular and molecular processes associated with LAM and highlight emerging treatments.</div></front></TEI><affiliations><list><country><li>Australie</li></country><region><li>Nouvelle-Galles du Sud</li></region><settlement><li>Sydney</li></settlement><orgName><li>Université de Sydney</li></orgName></list><tree><country name="Australie"><region name="Nouvelle-Galles du Sud"><name sortKey="Moir, Lyn M" sort="Moir, Lyn M" uniqKey="Moir L" first="Lyn M" last="Moir">Lyn M. 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