Pathology

Lymphangioleiomyomatosis Lam Diagnosis and Treatment



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Lymphangioleiomyomatosis (LAM) is a rare form of lung disease that mostly affects women in their 30s and 40s. Smooth muscle cells grow abnormally in the lungs. The cystic growth can block airways and the lungs usually develop pleural effusion (accumulation of fluid in the lung space). This can cause difficulty in breathing. When there is obstruction in the air pathways, the lungs will have difficulty in supplying oxygen to the rest of the body. Most patients with LAM have collapsed lungs (pneumothorax). Others have growth in their kidneys and other organs.

Diagnosing LAM can be difficult because its symptoms are similar to asthma, emphysema and other lung diseases. Because of this, a patient is diagnosed with another disease before LAM is discovered. Initially, lung function tests, X-rays are administered.  However, these tests may not confirm LAM. Lung biopsy can detect the presence of LAM. It is done by insert a needle in the chest to extract tissue sample from the lung. Other procedure used is transbronchial biopsy. A long, thing tube with light in the end is inserted to the nose or mouth and it passes to the trachea to reach the lungs where a tissue sample can be collected. The sample will be tested using HMB45 staining process. HMB45 is an antibody used as a marker for melanoma. Studies have shown that tissues from patients with LAM (specifically pulmonary LAM) show some immune reaction to HMB45. However, the more effective way of detecting LAM is done through high-resolution CT scan. It can show a more detailed picture of the lung tissues. It is less invasive and painless.

The cause of this disease is not yet known and the cure is not yet discovered. Instead, there are supportive treatments that will help ease the symptoms. Bronchodilators can help breathlessness. These medications help open up airways to allow normal breathing. Inhalers used by asthmatic patients are being used by some LAM patients. Pleural effusion can be removed through thoracentensis. A tube is inserted in the chest to remove fluid from the lungs. A diet that is low in fat can help prevent fluid accumulation in the lungs. Oxygen therapy can be used for those patients with low levels of oxygen. A surgical procedure called pleurodesis can be done to prevent recurrence of pneumothorax (collapsed lungs) and pleural effusion. The procedure will stick together linings in the lungs. It removes spaces between linings and prevents the accumulation of fluid.  Undergoing lung transplant is an option for patients with severe LAM. This is usually used as a last resort to LAM patients especially when they become dependent on oxygen.

A study published in The New England Journal of Medicine shows that sirolimus has some beneficial effects on LAM patients. Sirolimus is a drug used by transplant patients to fight organ rejection. The same drug shows improvement in lung functions in LAM patients. Several studies are still progress to understand this disease more.


Sources:
http://www.lunguk.org/you-and-your-lungs/conditions-and-diseases/lymphangioleiomyomatosis-lam
http://www.nhlbi.nih.gov/health/dci/Diseases/lam/lam_whatis.html
http://www.bmj.com/content/340/bmj.c848.full
http://erj.ersjournals.com/content/8/11/1879.full.pdf

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