Pathology

Causes and Symptoms of Lymphangioleiomyomatosis Lam



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Lymphangioleiomyomatosis (LAM) is an unusual invasion of a type of muscle cell into the lungs, the airways, blood and lymph vessels. These LAM cells have been found in kidneys as well. They cause cystic destruction of the lungs with progressive pulmonary dysfunction. Tumors may even present themselves in the abdominal region. 

In the United States alone, over 500 known cases exist, while LAM has also been found in Europe and Japan. LAM patients can survive a decade, with the mortality of 8.5 years being the average number of years for 78% of known patients. Because it is frequently misdiagnosed as asthma and chronic obstructive pulmonary disease, LAM may remain undetected in its initial stages.

While LAM has no race preference, premenopausal women are likely to develop this condition, and female hormonal malfunctions could be involved in causing the phenomena. Known female patients of LAM undergo therapies to antagonize the effects of estrogen, these therapies have not been proven in their effectiveness to positively control or eradicate the presence of LAM cells.

Men who fall victims to LAM are likely sufferers of tuberous sclerosis complex (TSC). LAM was first reported in a TSC male patient in 1918 by Lutembacher R. Dysembryomes. TSC is an inherited disorder that shares features with LAM, making LAM in males difficult to detect especially when it is TSCis an inherited disorder. Because LAM occurs in TSC patients, there is a remote possibility that LAM is also an inherited disorder.

While little is known about the causes of LAM other than a remote possibility of inherited genes, a relation to TSC, and in women a possibility of high ER states suggesting hormonal influences, symptoms of LAM are more easily detected, although they are also common to other diseases, and may lead to delayed confirmation of LAM in a patient.

According to the LAM Foundation, common symptoms of LAM are:

* Shortness of breath, especially after exertion. Because shortness of breath often occurs after exertion, usually after strenuous exercise, it is likely to be mistaken to be the result of the exercise, and not LAM. However, if the shortness of breath is prevalent in the absence of strenuous activities, it is best to go for a check up. 

* Frequent coughs. Coughs are symptoms of many other ailments. In the case of fluctuating weather, it is more likely for this symptom to be linked to the common cough and cold than to LAM. It is thus important that coughs are looked into by medical personnel such as a family doctor, and tracked, instead of self-medicating or hopping from one doctor to another if the cough prevails.  

* Small amounts of blood being coughed out. The presence of blood in coughs is neer a good thing, even if it is related to the common Influenza. Have your present medical condition checked again, even if you have seen the doctor a couple of times recently. If the usual cough medication has not worked, the cough is likely to be a symptom of a more serious condition.  

* Fatigue. If you usually do not feel fatigue but do so, especially if you are a woman in pre-menopausal stages, consult a doctor or gynaecologist. Even if you do not have LAM, you may have other medical conditions related to the onslaught of menopause that needs medical attention. It is better to err on the safe side than risk having LAM or other diseases go undetected until the later irreversible stages. 

* Chest pain, caused by the collapse of one or both lungs. At this stage, if LAM is present, it is likely to be prevalent. Other possible symptoms at this stage are the build-up of fluid in the lungs and extreme fatigue.

* Enlarged lymph nodes. LAM affects the lymphs and enlarged lymph nodes are possible symptoms of the disease.

Crackles, clubbing, wheezes, pleural effusion and Pneumothorax Ascites are other less known symptoms, according to Joel Moss.

It is likely for two or more of the symptoms to appear together. Because these symptoms are also prevalent in sufferers of other diseases such as Asthma, Emphysema, Chronic Bronchitis, TSC, and Pneumonia, LAM may not be diagnosed until a later stage.

The presence of LAM in patients is best detected and confirmed with chest X-Rays and high resolution CT scans. These will show up unnatural cell formation, layers of cells, cysts and lesions. Lung biopsy, the extraction and study of these unnatural cell growths, will confirm the presence of LAM or other diseases.

Blood tests can only determine if sufficient oxygen is the lungs are providing sufficient oxygen to the bloodstream but cannot detect the presence of LAM cells. Similarly, lung function tests can only show up the effect of LAM on how well the lungs are functioning, but not confirm the presence of LAM.

To date, because causes of LAM are evasive, and symptoms are predominant in other ailments, LAM may remain undetected until a later stage. It is therefore safer to go for X-rays and CT scans if external symptoms such as coughs, fatigue and shortness of breath occur together for a longer than normal period of time. Even if it is not LAM, another ailment that needs medical attention may prevail.

Reference:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1682009/

http://www.lunguk.org/you-and-your-lungs/conditions-and-diseases/lymphangioleiomyomatosis-lam

http://emedicine.medscape.com/article/299545-overview

http://www.thelamfoundation.org/

http://en.wikipedia.org/wiki/Lymphangioleiomyomatosis



  

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  • InfoBoxCallToAction ActionArrowhttp://www.thelamfoundation.org/about-lam/symptoms-of-lam.html
  • InfoBoxCallToAction ActionArrowhttp://emedicine.medscape.com/article/299545-overview
  • InfoBoxCallToAction ActionArrowhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC1682009/
  • InfoBoxCallToAction ActionArrowhttp://www.lunguk.org/you-and-your-lungs/conditions-and-diseases/lymphangioleiomyomatosis-lam
  • InfoBoxCallToAction ActionArrowhttp://emedicine.medscape.com/article/299545-overview
  • InfoBoxCallToAction ActionArrowhttp://www.thelamfoundation.org/
  • InfoBoxCallToAction ActionArrowhttp://en.wikipedia.org/wiki/Lymphangioleiomyomatosis