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http://www.lung.ca/diseases-maladies/a-z/lymphangioleiomyomatosis-ly...
Diseases AZ
Lymphangioleiomyomatosis (LAM)
What is Lymphangioleiomyomatosis?
Lymphangioleiomyomatosis (LAM) is a rare lung disease that was first described in the medical literature by von Stossel in 1937. The disease is characterized by an unusual type of muscle cell that invades the tissue of the lungs, including the airways, and blood and lymph vessels. Over time, these muscle cells form into bundles and grow into the walls of the airways, and blood and lymph vessels, causing them to become obstructed. Although these cells are not considered cancerous, they act somewhat like cancer cells in that they grow uncontrollably throughout the lung. Over time, the muscle cells block the flow of air, blood, and lymph to and from the lungs, preventing the lungs from providing oxygen to the rest of the body. Kidney tumors that are often asymptomatic may also be found in patients with LAM. Lymphangioleiomyomatosis is pronounced lim - fan'- je - o - Li'- o - mi'- o - ma - to'- sis. Lymph and angio refer to the lymph and blood vessels. Leiomyomatosis refers to the formation of the bundles of the unusual muscle cells. The cause of LAM is not known. However, a recent paper reports that tuberous sclerosis gene mutations are a cause of lymphyangioleiomyomatosis (LAM). The mutations were found in the angiomyolipoma cells and LAM cells from four women with LAM. The mutations were not present in normal lung, kidney or blood cells, indicating that these women with LAM do not have the inherited disease, tuberous sclerosis. Identifying this genetic link between tuberous sclerosis and sporadic LAM is an important step in LAM research. [Ref: Mutations in the tubersous sclerosis complex gene TSC2 are a cause of sporatic lymphyangioleiomyomatosis; Carsillo, Astrinidis and Henske; PNAS 2000 97:6085-90.]
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http://www.lung.ca/diseases-maladies/a-z/lymphangioleiomyomatosis-ly...
(pleural effusion). The fluid may be straw-colored (lymph), or fat-containing, milky white (chyle), or pinkish-red if it contains blood. A physician can remove some of this fluid with a needle and syringe to deteremine its composition and origin. If large amounts of this fluid accumulate in the chest cavity, it may have to be removed through a tube surgically inserted into the chest. It is estimated that 30 to 50 percent of LAM patients will develop leakage of air into the chest cavity (pneumothorax), and up to 80 percent will have leakage of fluid into the chest cavity (pleural effusions). Coughing up blood-stained sputum or blood (hemoptysis) occurs less frequently.
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http://www.lung.ca/diseases-maladies/a-z/lymphangioleiomyomatosis-ly...
(bronchoscope) is inserted down the windpipe (trachea), and into the lungs. Bits of lung tissue are sampled, using a tiny forceps. This procedure is usually done in a hospital on an outpatient basis under local anesthesia. It is less reliable than an open lung biopsy because the amount of tissue that can be sampled is sometimes inadequate for diagnostic studies. After the lung tissue is removed, it is examined in a pathology laboratory for the presence of the abnormal muscle cells and cystic changes characteristic of LAM.
2012 Canadian Lung Association. All Rights Reserved. Comments or questions? Last updated on September 24, 2012
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