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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Lymphangioleiomyomatosis


Other Names for this Disease
  • LAM
  • Lymphangio-myomatosis
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Overview


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.[1]

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:[1]
  • Growths in other organs, including the liver and brain
  • Large tumors on their lymph nodes

  • There are two forms of LAM:[1]
  • 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.
  • Last updated: 7/16/2013

    References

    1. What is LAM?. National Heart Lung and Blood Institute (NHLBI). 2006; http://www.nhlbi.nih.gov/health/dci/Diseases/lam/lam_whatis.html. Accessed 2/13/2008.
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    Basic Information

    • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

    In Depth Information

    • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
    • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
    • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
    • PubMed is a searchable database of medical literature and lists journal articles that discuss Lymphangioleiomyomatosis. Click on the link to view a sample search on this topic.