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 Large tumors on their lymph nodes
There are two forms of LAM: Sporadic LAM, which occurs for unknown reasons 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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General Support Organizations

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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 166 free, full-text research articles on human participants. First 3 results:

Rapamycin-insensitive up-regulation of adipocyte phospholipase A2 in tuberous sclerosis and lymphangioleiomyomatosis.
 

Author(s): Chenggang Li, Erik Zhang, Yang Sun, Po-Shun Lee, Yongzhong Zhan, Yanan Guo, Juan C Osorio, Ivan O Rosas, Kai-Feng Xu, David J Kwiatkowski, Jane J Yu

Journal:

 

Tuberous sclerosis syndrome (TSC) is an autosomal dominant tumor suppressor gene syndrome affecting multiple organs, including renal angiomyolipomas and pulmonary lymphangioleiomyomatosis (LAM). LAM is a female-predominant interstitial lung disease characterized by the progressive ...

Last Updated: 28 Oct 2014

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Natural history of angiomyolipoma in lymphangioleiomyomatosis: implications for screening and surveillance.
 

Author(s): Zhao W Yeoh, Vidya Navaratnam, Rupesh Bhatt, Ian McCafferty, Richard B Hubbard, Simon R Johnson

Journal:

 

LAM is a rare disease of women categorised by lung cysts and lymphatic abnormalities. The disease occurs sporadically or associated with Tuberous Sclerosis Complex (TSC-LAM). Angiomyolipoma, a benign tumour, prone to haemorrhage, occurs mostly in the kidneys in many of these patients. ...

Last Updated: 22 Oct 2014

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Incidental detection of retroperitoneal lymphangioleiomyomatosis (LAM) - CT and MRI findings with relevance to the urologist.
 

Author(s): Chandan Phukan, Shailesh Prabhu, Vivek Venkatramani

Journal: Int Braz J Urol. ;40(4):574-5.

 

Last Updated: 25 Sep 2014

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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 28 free, full-text review articles on human participants. First 3 results:

Towards personalised therapy for lymphangioleiomyomatosis: lessons from cancer.
 

Author(s): Souheil El-Chemaly, Elizabeth P Henske

Journal: Eur Respir Rev. 2014 Mar;23(131):30-5.

 

Lymphangioleiomyomatosis (LAM) is a rare cystic, destructive lung disease occurring almost exclusively in females. Bi-allelic inactivating tuberous sclerosis complex (TSC) gene mutations occur in LAM cells, resulting in activation of the mTORC1 pathway. Pivotal clinical trials have ...

Last Updated: 4 Mar 2014

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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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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

National Lymphangioleiomyomatosis Registry, France
 

Status: Recruiting

Condition Summary: Lymphangioleiomyomatosis

 

Last Updated: 16 Oct 2013

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Nebulized or Inhaled Albuterol for Lymphangioleiomyomatosis
 

Status: Recruiting

Condition Summary: Lymphangioleiomyomatosis

 

Last Updated: 8 Nov 2014

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Trial of Aromatase Inhibition in Lymphangioleiomyomatosis
 

Status: Recruiting

Condition Summary: Lymphangioleiomyomatosis

 

Last Updated: 10 Jun 2011

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