Lymphangioleiomyomatosis - LAM

Lymphangioleiomyomatosis or LAM is a lung disease characterized by a strange type of muscle cell that infests the tissue of the lungs, including the airways, blood vessels, 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 blocked. Although these cells are not believed malignant, they act somewhat like cancer cells in that they grow unrestrained throughout the lung. The muscle cells in time block the flow of air, blood, and lymph to and from the lungs, preventing the lungs from supplying oxygen to the rest of the body.

The proliferating growth that occurs in the type of LAM seen in patients with tuberous sclerosis (TSC-LAM) has been shown to represent clones of the smooth muscle in those patients' renal angiomyolipomas. Thus it is believed to represent metastases of this "benign" tumor. There is a female preponderance to TSC-LAM.

To date, more than 500 cases exist in the United States. As the disease becomes better recognized because of increased awareness and better diagnostic techniques, the prevalence may increase.

Exact data on survival rates are hard to gather because LAM is often misdiagnosed as asthma or other more common maladies, and may not be rightly discovered until it is in an advanced condition. An inclusive study of all known British LAM patients found that out of 21 patients that had been noticed for 15 years or more since diagnosis, 18 were still alive; and 11 of 12 patients that had been observed for 20 years or more than that were alive.

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Posted byParvez Ahmed at 8:35 PM  

1 comments:

Anonymous said... November 19, 2008 at 6:22 AM  

Very informative article, thank you.

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