Lymphangioleiomyomatosis

Common Name(s)

Lymphangioleiomyomatosis, Lymphangioleiomyomatosis (LAM)

"Lymphangioleiomyomatosis (lim-FAN-je-o-LI-o-MI-o-ma-TO-sis), or LAM, is a rare lung disease that mostly affects women in their mid-forties. In LAM, an unusual type of cell begins to grow out of control throughout the body, including in the lungs, lymph nodes and vessels, and kidneys. Over time, these LAM cells form cysts and clusters of cells, which grow throughout the lungs and slowly block the airways. They also destroy the normal lung tissue and replace it with cysts. As a result, air cannot move freely in and out of the lungs, and the lungs cannot supply enough oxygen to the body’s other organs.

More than 1 out of every 3 people with LAM also develops growths called angiomyolipomas, or AMLs, in their kidneys. People with LAM also may develop growths in other organs, including the liver and brain, and large tumors on their lymph nodes.
There are two forms of LAM: sporadic LAM, which occurs for unknown reasons and LAM that occurs in people with a rare inherited disease called tuberous sclerosis complex. This is often a milder form.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Lymphangioleiomyomatosis" for support, advocacy or research.

LAM Foundation

The LAM Foundation urgently seeks safe and effective treatments and ultimately a cure for LAM through advocacy and the funding of promising research. We are dedicated to serving the scientific, medical and patient communities by offering information, resources and a worldwide network of hope and support.

Last Updated: 5 Mar 2013

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LAM Treatment Alliance

The LAM Treatment Alliance (LTA) is a 501(c)(3) non-profit organization fostering innovative patient engagement and awareness of wellness within the context of LAM. The LTA provides tools, access, and information to patients in an effort to improve health, quality of life, and modulation of disease.

Last Updated: 13 Mar 2013

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Lymphangioleiomyomatosis" for support, advocacy or research.

LAM Foundation

The LAM Foundation urgently seeks safe and effective treatments and ultimately a cure for LAM through advocacy and the funding of promising research. We are dedicated to serving the scientific, medical and patient communities by offering information, resources and a worldwide network of hope and support.

http://www.thelamfoundation.org

Last Updated: 5 Mar 2013

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LAM Treatment Alliance

The LAM Treatment Alliance (LTA) is a 501(c)(3) non-profit organization fostering innovative patient engagement and awareness of wellness within the context of LAM. The LTA provides tools, access, and information to patients in an effort to improve health, quality of life, and modulation of disease.

http://www.curelam.org

Last Updated: 13 Mar 2013

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Lymphangioleiomyomatosis" returned 151 free, full-text research articles on human participants. First 3 results:

Integration of mTOR and estrogen-ERK2 signaling in lymphangioleiomyomatosis pathogenesis.
 

Author(s): Xiaoxiao Gu, Jane J Yu, Didem Ilter, Nickolas Blenis, Elizabeth Petri Henske, John Blenis

Journal: Proc. Natl. Acad. Sci. U.S.A.. 2013 Sep;110(37):14960-5.

 

Lymphangioleiomyomatosis (LAM) is a destructive lung disease of women associated with the metastasis of tuberin-null cells with hyperactive mammalian target of rapamycin complex 1 (mTORC1) activity. Clinical trials with the mTORC1 inhibitor rapamycin have revealed partial efficacy ...

Last Updated: 11 Sep 2013

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Lymphatic endothelial differentiation in pulmonary lymphangioleiomyomatosis cells.
 

Author(s): Jennifer M Davis, Elizabeth Hyjek, Aliya N Husain, Le Shen, Jennifer Jones, Lucia A Schuger

Journal: J. Histochem. Cytochem.. 2013 Aug;61(8):580-90.

 

Pulmonary lymphangioleiomyomatosis (LAM) is a rare, low-grade neoplasm affecting almost exclusively women of childbearing age. LAM belongs to the family of perivascular epithelioid cell tumors, characterized by spindle and epithelioid cells with smooth muscle and melanocytic differentiation. ...

Last Updated: 26 Jul 2013

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MicroRNA-21 is induced by rapamycin in a model of tuberous sclerosis (TSC) and lymphangioleiomyomatosis (LAM).
 

Author(s): Anil J Trindade, Douglas A Medvetz, Nicole A Neuman, Faina Myachina, Jane Yu, Carmen Priolo, Elizabeth P Henske

Journal: PLoS ONE. 2013 ;8(3):e60014.

 

Lymphangioleiomyomatosis (LAM), a multisystem disease of women, is manifest by the proliferation of smooth muscle-like cells in the lung resulting in cystic lung destruction. Women with LAM can also develop renal angiomyolipomas. LAM is caused by mutations in the tuberous sclerosis ...

Last Updated: 4 Apr 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Lymphangioleiomyomatosis" returned 27 free, full-text review articles on human participants. First 3 results:

LAM cells biology and lymphangioleiomyomatosis.
 

Author(s): Irmina Grzegorek, Katarzyna Drozdz, Marzenna Podhorska-Okolow, Andrzej Szuba, Piotr Dziegiel

Journal: Folia Histochem. Cytobiol.. 2013 ;51(1):1-10.

 

Progressive lung tissue destruction in lymphangioleiomyomatosis (LAM) occurs as a result of excessive proliferation of LAM cells caused by a mutation in one of the tuberous sclerosis complex suppressor genes, TSC1 or TSC2. These cells show constitutive activation of the mammalian ...

Last Updated: 21 May 2013

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Targeted approaches toward understanding and treating pulmonary lymphangioleiomyomatosis (LAM).
 

Author(s): Stephen R Hammes, Vera P Krymskaya

Journal: Horm Cancer. 2013 Apr;4(2):70-7.

 

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease found almost exclusively in women that is characterized by neoplastic growth of atypical smooth muscle-like cells in the lung, destruction of lung parenchyma, and obstruction of lymphatics. These processes lead to the formation ...

Last Updated: 25 Feb 2013

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Lymphangioleiomyomatosis - a wolf in sheep's clothing.
 

Author(s): Elizabeth P Henske, Francis X McCormack

Journal: J. Clin. Invest.. 2012 Nov;122(11):3807-16.

 

Lymphangioleiomyomatosis (LAM) is a rare progressive lung disease of women. LAM is caused by mutations in the tuberous sclerosis genes, resulting in activation of the mTOR complex 1 signaling network. Over the past 11 years, there has been remarkable progress in the understanding ...

Last Updated: 1 Nov 2012

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Pulmonary Rehabilitation in Lymphangioleiomyomatosis
 

Status: Recruiting

Condition Summary: Lymphangioleiomyomatosis

 

Last Updated: 8 Dec 2013

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National Lymphangioleiomyomatosis Registry, France
 

Status: Recruiting

Condition Summary: Lymphangioleiomyomatosis

 

Last Updated: 16 Oct 2013

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The Tolerability of Saracatinib in Subjects With Lymphangioleiomyomatosis (LAM) (SLAM-1)
 

Status: Recruiting

Condition Summary: Pulmonary Lymphangioleiomyomatosis

 

Last Updated: 17 Apr 2014

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