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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



What is lymphangioleiomyomatosis (LAM)?

How is lymphangioleiomyomatosis (LAM) treated?


What is 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.[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

    How is lymphangioleiomyomatosis (LAM) treated?

    While there is currently no cure for LAM, research has led to major progress in the treatment of symptoms, and diagnosing and understanding LAM. There are a number of treatments that may relieve symptoms or prevent complications from the disease. Treatments vary from patient to patient, depending on the severity of the disease. No all patients have the same response to treatment.[2][3]

  • Medicines (e.g., diuretics, hormone therapy, bronchodilators that relax the muscles around the airways)
  • Oxygen therapy - as lung capacity declines, supplemental or full-time oxygen therapy may become necessary
  • Sirolimus therapy - Rapamycin (sirolimus) blocks the pathway that is affected in LAM cells, which helps stop their uncontrolled growth. The use of sirolimus therapy may be considered in patients with moderate to severe LAM, or those with progressive disease.
  • Procedures to remove air or fluid from the chest or abdominal cavities and prevent it from building up again
  • Procedures to remove angiomyolipoma (AML), or benign kidney tumors
  • Lung transplantation - a procedure to replace one or both lungs, this should be considered as a last resort due to the risk of serious adverse reactions, including major bleeding, pneumonia, pulmonary edema, long-term infections, and possibly painful scarring.
  • Since LAM occurs almost exclusively in women of reproductive age, researchers believe the hormone estrogen might be involved in the abnormal muscle cell growth that characterizes the disease. Although there is no direct evidence that there is a relationship between estrogen and LAM, the treatment of LAM has focused on reducing the production or effects of estrogen. This could include estrogen or other hormone suppressing drugs. Additionally, doctors believe pregnancy may accelerate the progression of LAM. Women with LAM are urged to speak with a health care professional before getting pregnant.[3]

    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.
    2. How Is LAM Treated?. National Heart Lung and Blood Institute (NHLBI). 2006; http://www.nhlbi.nih.gov/health/dci/Diseases/lam/lam_treatments.html. Accessed 2/15/2008.
    3. Treating LAM. The LAM Foundation. 2007; http://www.thelamfoundation.org/medical-providers/lam-therapy. Accessed 2/15/2008.