Diffuse Pulmonary Lymphangiomatosis

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It is possible that the main title of the report Diffuse Pulmonary Lymphangiomatosis is not the name you expected.

Disorder Subdivisions

  • None

General Discussion


Diffuse pulmonary lymphangiomatosis is a disease in which the overgrowth (proliferation) of lymphatic vessels (lymphangiomatosis) occurs in the lungs, pleura and typically the surrounding soft tissue of the chest (mediastinum). Lymphatic vessels are part of the lymphatic system, which includes lymph nodes, the small nodules where certain white blood cells (lymphocytes) and other cells participate in the immune regulatory system of the body. When fluid leaves arteries and enters the soft tissue and organs of the body, it does so without red or white blood cells. This thin watery fluid is known as lymph. The lymphatic system consists of a network of tubular channels (lymph vessels) that transport lymph back into the bloodstream. Lymph accumulates between tissue cells and contains proteins, fats, and lymphocytes. As lymph moves through the lymphatic system, it passes through the network of lymph nodes that help the body to deactivate sources of infection (e.g., viruses, bacteria, etc.) and other potentially injurious substances and toxins. Groups of lymph nodes are located throughout the body, including in the neck, under the arms (axillae), at the elbows, and in the chest, abdomen, and groin. The lymphatic system also includes the spleen, which filters worn-out red blood cells and produces lymphocytes; and bone marrow, which is the spongy tissue inside the cavities of bones that manufactures blood cells.

Lymphangiomatosis can potentially affect any part of the body except the brain. The disorder can be widespread, affecting multiple areas simultaneously, as in the case of diffuse pulmonary lymphangiomatosis, or be isolated to one area (i.e. the lungs and chest). The specific symptoms and severity vary, depending in part upon the size and the specific location of the abnormalities. Diffuse pulmonary lymphangiomatosis causes functional impairment of the lungs and when the chest wall is involved, may be associated with disfigurement. The exact cause of diffuse pulmonary lymphangiomatosis is unknown.


There is a lack of consensus among the medical community as to the proper terminology for disorders and malformations associated with lymphatics. In general, however, a lymphangioma is a relatively localized collection of abnormal lymphatic vessels, lymphangiectasis refers to dilatation of lymphatics and lymphangiomatosis refers to an increase in lymphatic number. In some patients there is overlap between these and so exact classification becomes problematic. Lymphangiomatosis may also occur in association with better-characterized disease such as Gorham's disease. Gorham's disease is a form of lymphangiomatosis in which the lymphatics proliferate in bone, resulting in progressive bone loss. Another disease, which has a similar sounding name, is lymphangioleiomyomatosis, a distinct disorder caused by proliferation of smooth muscle-like cells that, despite the similarity in the names, is unrelated to lymphangiomatosis.

NORD has separate reports on lymphatic malformations, Gorham's disease, lymphangioleiomyomatosis, and several types of lymphangiectasia. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database.). Lymphatic disorders are a rapidly growing disease family and information about these disorders and the terminology used to describe them are constantly changing. Physicians and researchers are working to create a standardized classification and nomenclature system for these disorders.

Supporting Organizations

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
Website: http://rarediseases.info.nih.gov/GARD/

Lymphangiomatosis & Gorham's Disease Alliance, Inc. (LGDA)

19919 Villa Lante Place
Boca Raton, FL 33434
Tel: (561)441-9766
Email: info@lgdalliance.org
Website: http://www.lgdalliance.org/

Lymphatic Education & Research Network

261 Madison Avenue
9th Floor
New York, NY 10016
Tel: 516-625-9675
Fax: 516-625-9410
Email: LERN@LymphaticNetwork.org
Website: http://www.lymphaticnetwork.org

Lymphatic Malformation Institute

7475 Wisconsin Ave
Suite 600
Bethesda, MD 20814
Email: ngarg@lmiresearch.org
Website: http://www.lmiresearch.org

National Organization of Vascular Anomalies

PO Box 38216
Greensboro, NC 27438-8216
Email: admin@mail.novanews.org
Website: http://www.novanews.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

Last Updated:  3/23/2016
Copyright  2013 National Organization for Rare Disorders, Inc.