Pathology

Lymphangioleiomyomat Osis Lam Diagnosis and Treatment



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Lymphangioleiomyomatosis is a rare medical disorder that affects lungs, kidneys and axial lymphatics.  Also known as LAM, this disease is characterized by cystic lung changes, angiomyolipomas and enlargement of the abdominal and pelvic lymphatics.  LAM is a progressive disease, which leads to respiratory failure and death.  This medical condition was first reported in 1918 by Lutembacher.  

The exact cause of this disease is unknown.  But it is observed that women (between menarche and menopause) are more prone to this disease.  Patients with tuberous sclerosis (TSC) are more likely to be affected by LAM.  Chest pain, frequent cough, wheezing, shortness of breath (dyspnea), blood in urine, painful swelling in the abdomen, enlarged lymph nodes, pneumothorax and pleural effusions are signs and symptoms of Lymphangioleiomyomatosis.  

Technological advances have made it possible to diagnose this disease at its earlier stages.  But there are no universally agreed criteria for the diagnosis of this medical condition.  Pulmonologists check for proper functioning of the lungs.  Instruments like spirometer help in identifying erratic breathing.  Blood tests help doctors get an idea about blood chemistry and blood cells.  Pulse oximetry is very useful in diagnosis of LAM.  This test gives an estimate of the amount of oxygen in the blood stream of the patient.  

High Resolution CT Scan (HRCT scan) is generally used for diagnosing LAM.  The computer generated image of the lungs clearly reveals shadows of cell clusters, cysts, collapsed lung, excess fluid and enlarged lymph nodes.  HRCT scan also reveals how much normal lung tissue has been replaced by the LAM cysts.  Abdominal and pelvic HRCT scans help in identifying growth of muscle cells in kidneys, abdomen and lymph nodes.  Chest x ray is also used to diagnose this medical condition.  But it is not as effective as HRCT scan.  

Doctors may also use Video Assisted Thoracoscopy (VAT) to diagnose this disease.  This procedure enables the doctor to look inside the chest.  Doctors prefer VAT over open lung biopsy.  LAM can also be diagnosed using transbronchial biopsy.  Patients diagnosed with sporadic LAM may be advised to have a CT scan or MRI scan of the head.  These tests help in identifying TSC.  

There are no proven treatments for LAM.  It is also not clear whether early treatment prevents progression of the disease.  Treatment is mainly supportive.  Doctors generally prescribe bronchodilators.   These medicines relax the muscles around the airways.  This improves breathing.  Oxygen therapy may be used in some cases.  Lung transplantation may also be advised.  Calcium and vitamin D supplements may be prescribed in an endeavor to prevent osteoporosis.  

Doctors use procedures like thoracentesis and paracentesis to remove fluid from the body.  In some cases a needle and syringe may be used for the same purpose.  Air is removed from the chest in order to provide relief from shortness of breath.  Surgery may be performed to remove angiomyolipoma (AML).  Hormone therapy may be used in some cases to treat LAM.  This is done to limit the effects of estrogen on the body.  Rapamycin is found to be effective in shrinking tumors in the kidneys of women affected by LAM.  Unfortunately this medicine has many side effects.     

Patients with symptoms of this disease should be taken to a certified pulmonologist for further medical care.  There is need to create more awareness about LAM.  

References:

http://www.thelamfoundation.org/

http://www.medterms.com/script/main/art.asp?articlekey=19212

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

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

EW56Bhttp://www.nhlbi.nih.gov/health/dci/Diseases/lam/lam_treatments.htmlWH6PDhttp://www.nhlbi.nih.gov/health/dci/Diseases/lam/lam_treatments.htmlZ6EFP

http://www.orpha.net/data/patho/GB/uk-LAM.pdf

http://rarediseases.about.com/od/rarediseasesl/a/lam.htm

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ARTICLE SOURCES AND CITATIONS
  • InfoBoxCallToAction ActionArrowhttp://www.thelamfoundation.org/
  • InfoBoxCallToAction ActionArrowhttp://www.medterms.com/script/main/art.asp?articlekey=19212
  • InfoBoxCallToAction ActionArrowhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC1682009/
  • InfoBoxCallToAction ActionArrowhttp://emedicine.medscape.com/article/299545-overview
  • InfoBoxCallToAction ActionArrowhttp://www.nhlbi.nih.gov/health/dci/Diseases/lam/lam_treatments.html
  • InfoBoxCallToAction ActionArrowhttp://www.orpha.net/data/patho/GB/uk-LAM.pdf
  • InfoBoxCallToAction ActionArrowhttp://rarediseases.about.com/od/rarediseasesl/a/lam.htm