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 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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The 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: 3 Mar 2016

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General Support Organizations

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

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

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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The 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: 3 Mar 2016

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General Support Organizations

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

Treatment of lymphangioleiomyomatosis and Camões.
 

Author(s): Rogério Rufino

Journal: J Bras Pneumol. ;41(4):295-6.

 

Last Updated: 24 Sep 2015

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Pulmonary lymphangioleiomyomatosis--a case report.
 

Author(s): Chun-Jie Li, Guo-Zheng Gao, Meilin Xu, Guan-Wei Fan

Journal: J Cancer Res Ther. 2015 Aug;11 Suppl 1():C138-9.

 

Pulmonary lymphangioleiomyomatosis (PLAM) is a rare disease, occurs in 16-68-year-old women, especially in women of childbearing age. High-resolution computed tomography would be useful for diagnosis of PLAM. Immunohistochemistry of smooth muscle actin (SMA) and HMB-45 smooth muscle ...

Last Updated: 1 Sep 2015

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Development of ATAQ-LAM: a tool to assess quality of life in Lymphangioleiomyomatosis.
 

Author(s): Tarik D Walker, Jennifer Desserich, Karen Albright, Frederick S Wamboldt, Amanda Belkin, Kaitlin Fier, Jeffrey J Swigris

Journal:

 

Lymphangioleiomyomatosis (LAM) is a progressive lung disease that impairs health-related quality of life (HRQL).

Last Updated: 29 Jul 2015

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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 30 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 Genetics Home Reference.

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: 11 May 2016

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Characterization of Patients With Tuberous Sclerosis Complex, Lymphangioleiomyomatosis and Angiomyolipoma
 

Status: Not yet recruiting

Condition Summary: Tuberous Sclerosis; Lymphangioleiomyomatosis; Angiomyolipoma

 

Last Updated: 24 Aug 2015

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