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

Protein-losing Enteropathy Caused by Intestinal or Colonic Lymphangiectasia Complicated by Sporadic Lymphangioleiomyomatosis: A Report of Two Cases.
 

Author(s): Koichi Nishino, Kaku Yoshimi, Tomoyoshi Shibuya, Takuo Hayashi, Keiko Mitani, Etsuko Kobayashi, Masako Ichikawa, Tetsuhiko Asao, Yohei Suzuki, Tadashi Sato, Satomi Shiota, Yuzo Kodama, Kazuhisa Takahashi, Kuniaki Seyama

Journal: Intern. Med.. 2017 ;56(8):943-948.

 

This report describes two patients with sporadic lymphangioleiomyomatosis complicated by protein-losing enteropathy (PLE). Imaging studies indicated retroperitoneal lymphangioleiomyomas and abnormalities of the adjacent digestive tract. Endoscopic mucosal biopsy revealed colonic lymphangiectasia ...

Last Updated: 19 Apr 2017

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[Diagnosis and treatment for 2 cases of tuberous sclerosis complex with pulmonary lymphangioleiomyomatosis].
 

Author(s): Dandan Zong, Caihong Liu, Zhihui Shi, Li Zhou, Yating Peng, Ruoyun Ouyang

Journal: Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2016 Jul;41(7):771-6.

 

To improve the diagnosis and treatment for tuberous sclerosis complex (TSC) with pulmonary lymphangioleiomyomatosis, a retrospective analysis was performed based on the clinical data of 2 patients with such disease. Both of them have typical thin-walled cystic lesion throughout the ...

Last Updated: 5 Sep 2016

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

Pelvic lymphangioleiomyomatosis treated successfully with everolimus: Two case reports with literature review.
 

Author(s): Sharjil Wahid, Ping Chia Chiang, Hao Lun Luo, Shun-Chen Huang, Eing-Mei Tsai, Po Hui Chiang

Journal: Medicine (Baltimore). 2017 Mar;96(10):e4562.

 

Lymphangioleiomyomatosis (LAM) is a rare disease affecting young women caused by abnormal proliferation of smooth muscle-like cells (LAM cells) in the lungs and extrapulmonary sites (extrapulmonary LAM). The objective of this case series is to demonstrate marked regression in 2 cases ...

Last Updated: 8 Mar 2017

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The effect of mTOR inhibitors on respiratory infections in lymphangioleiomyomatosis.
 

Author(s): Andrew M Courtwright, Hilary J Goldberg, Elizabeth Petri Henske, Souheil El-Chemaly

Journal:

 

Lymphangioleiomyomatosis (LAM) is a destructive cystic lung disease. Mammalian target of rapamycin (mTOR) inhibitors are the primary treatment for LAM but it is unknown whether these immunosuppressing medications increase the risk for or the severity of respiratory infections in LAM ...

Last Updated: 18 Jan 2017

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The changing face of a rare disease: lymphangioleiomyomatosis.
 

Author(s): Sergio Harari, Olga Torre, Roberto Cassandro, Joel Moss

Journal: Eur. Respir. J.. 2015 Nov;46(5):1471-85.

 

Lymphangioleiomyomatosis is a rare disease characterised by cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects almost exclusively females and can occur sporadically or in patients with tuberous sclerosis complex. In the past decade remarkable ...

Last Updated: 2 Nov 2015

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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: 30 Jun 2017

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