Lymphangioleiomyomatosis

Common Name(s)

Lymphangioleiomyomatosis, Lymphangioleiomyomatosis (LAM)

Lymphangioleiomyomatosis (LAM) is a disorder that causes abnormal accumulations of cells as well as cysts, which are fluid-filled structures, in the lungs. LAM is caused by mutations in the TSC1 and TSC2 genes, which are involved in making proteins that help regulate the growth of certain cells. LAM cells are abnormal smooth muscle cells that grow and can block airways, blood vessels, and lymph vessels. In the lungs, these accumulations can block oxygen exchange to the blood, resulting in poor oxygen delivery to tissues. In addition to cyst development in the lungs, those affected by LAM often have kidney tumors that are benign or non-cancerous.

LAM almost always affects women. Symptoms usually begin around childbearing age. The overall average age of diagnosis is 48. LAM is a progressive disorder but develops at a various rates among different individuals. Symptoms include breathlessness and coughing up blood. A collapsed lung is often the first symptom women notice. LAM can be diagnosed by imaging of the lungs.

While there is currently no cure for LAM, there are treatment options to help manage the symptoms and prevent any complications. Treatments include drugs, oxygen treatment, removal of fluid from lungs, and lung transplant. If you have been diagnosed with LAM, talk to your doctor about the most current treatment options. In addition, a genetic counselor can help discuss inheritance and risks to other family members. Support groups are available for more resources and information.

Source: Advocacy organizations associated with the condition.

 

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

A Case of Successful Therapy by Intrapleural Injection of Fibrin Glue for Chylothorax after Lung Transplantation for Lymphangioleiomyomatosis.
 

Author(s): Hisashi Oishi, Yasushi Hoshikawa, Tetsu Sado, Tatsuaki Watanabe, Akira Sakurada, Takashi Kondo, Yoshinori Okada

Journal: Ann Thorac Cardiovasc Surg. 2017 Feb;23(1):40-44.

 

A 37-year-old woman underwent bilateral lung transplantation for lymphangioleiomyomatosis. Dense pleural adhesions due to past pleurodesis for chylothorax were observed and dissected in both thoracic cavities. The patient developed chylothorax after transplant. Chylothorax in the ...

Last Updated: 19 Jul 2016

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Two Kinds of Cystic Lung Lesions with Pulmonary Lymphangioleiomyomatosis in a Male.
 

Author(s): Sumitaka Yamanaka, Teruaki Mizobuchi, Masatoshi Kurihara

Journal: Ann Thorac Cardiovasc Surg. 2017 Feb;23(1):36-39.

 

A 34-year-old male with frequent recurrence of right pneumothorax was admitted to our hospital. He was a current smoker and outwardly male without genital aplasia. He was diagnosed as tuberous sclerosis complex (TSC) at 2 year-old and underwent transcatheter arterial embolization ...

Last Updated: 28 Jun 2016

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Study of breast cancer incidence in patients of lymphangioleiomyomatosis.
 

Author(s): Olivier Nuñez, Antonio Román, Simon R Johnson, Yoshikazu Inoue, Masaki Hirose, Álvaro Casanova, Gorka Ruiz de Garibay, Carmen Herranz, Gema Bueno-Moreno, Jacopo Boni, Francesca Mateo, Anna Petit, Fina Climent, Teresa Soler, August Vidal, José Vicente Sánchez-Mut, Manel Esteller, José Ignacio López, Nadia García, Anna Gumà, Raúl Ortega, María Jesús Plà, Miriam Campos, Emilio Ansótegui, María Molina-Molina, Claudia Valenzuela, Piedad Ussetti, Rosalía Laporta, Julio Ancochea, Antoni Xaubet, Marina Pollán, Miguel Angel Pujana

Journal: Breast Cancer Res. Treat.. 2016 Feb;156(1):195-201.

 

Molecular evidence has linked the pathophysiology of lymphangioleiomyomatosis (LAM) to that of metastatic breast cancer. Following on this observation, we assessed the association between LAM and subsequent breast cancer. An epidemiological study was carried out using three LAM country ...

Last Updated: 12 Mar 2016

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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 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: 22 Feb 2017

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A Study of Nintedanib for LymphAngioleioMyomatosis (LAM)
 

Status: Recruiting

Condition Summary: Lymphangioleiomyomatosis

 

Last Updated: 20 Feb 2017

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Nebulized or Inhaled Albuterol for Lymphangioleiomyomatosis
 

Status: Recruiting

Condition Summary: Lymphangioleiomyomatosis

 

Last Updated: 10 Mar 2017

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