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Alternative Names: Amyloid – primary
In medicine, amyloidosis refers to a variety of conditions in which amyloid proteins are abnormally deposited in organs and/or tissues. A protein is described as being amyloid if, due to an alteration in its secondary structure, it takes on a particular aggregated insoluble form similar to the beta-pleated sheet. Symptoms vary widely depending upon the site of amyloid deposition. Amyloidosis may be inherited or acquired.
The collection of these abnormal proteins interferes with the normal functioning of the organ affected.
Since there are more than 20 different proteins that may form amyloid, there are also many different types of amyloidosis.
Classification of amyloid:
The modern classification of amyloid disease tends to use an abbreviation of the protein that makes the majority of deposits, prefixed with the letter A. For example amyloidosis caused by transthyretin is termed “ATTR.” Deposition patterns vary between patients but are almost always composed of just one amyloidogenic protein. Deposition can be systemic (affecting many different organ systems) or organ-specific. Many amyloidoses are inherited, due to mutations in the precursor protein. Other forms are due to different diseases causing overabundant or abnormal protein production – such as with over production of immunoglobulin light chains in multiple myeloma (termed AL amyloid), or with continuous overproduction of acute phase proteins in chronic inflammation (which can lead to AA amyloid).
Out of the approximately 60 amyloid proteins that have been identified so far, at least 36 have been associated in some way with a human disease.
Amyloidosis is rare, being diagnosed in between one and five in every 100,000 people every year. It’s more common in older people and is also slightly more common in men than in women.
The cause of primary amyloidosis is unknown, but the condition is related to abnormal production of antibodies by a type of immune cell called plasma cells.
The symptoms depend on the organs affected by the deposits. These organs can include the tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys.
Primary amyloidosis can result in conditions that include:
•Carpal tunnel syndrome
•Gastrointestinal reflux (GERD)
•Heart muscle damage (cardiomyopathy)
The deposits build up in the affected organs, causing them to become stiff, which decreases their ability to function.
Risk factors have not been identified. Primary amyloidosis is rare. It is similar to multiple myeloma, and is treated the same way.
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•Irregular heart rhythm
•Numbness of hands and feet
•Shortness of breath
•Swelling in the arms and legs
•Weak hand grip
Additional symptoms that may be associated with this disease:
•Decreased urine output
•Hoarseness or changing voice
•Other tongue problems
Exams and Tests
Your doctor may discover that you have an enlarged liver or spleen.
If specific organ damage is suspected, your doctor may order tests to confirm amyloidosis of that organ. For example:
•Abdominal ultrasound may reveal a swollen liver or spleen.
•An abdominal fat pad biopsy, rectal mucosa biopsy, or a bone marrow biopsy can help confirm the diagnosis.
•A heart evaluation, including an ECG,may reveal arrhythmias, abnormal heart sounds, or signs of congestive heart failure. An echocardiogram shows poor motion of the heart wall, due to a stiff heart muscle.
•A carpal tunnel syndrome evaluation may show that hand grips are weak.Nerve conduction velocity shows abnormalities.
•Kidney function tests may show signs of kidney failure or too much protein in the urine ( nephrotic syndrome).
?BUN level is increased.
?Serum creatinine is increased.
?Urinalysis shows protein, casts, or fat bodies.
This disease may also alter the results of the following tests:
•Bence-Jones protein (quantitative)
•Carpal tunnel biopsy
•Immunoelectrophoresis – serum
It isn’t always easy to treat amyloidosis, and there is no treatment yet that specifically targets the amyloid depositing in the tissues. In cases where it’s secondary to another problem (AA amyloidosis), such as rheumatoid arthritis, treating that original problem may stop the progress of amyloidosis or may even reverse it.
In cases of primary amyloidosis (AL amyloidosis), chemotherapy drugs may be given to suppress production of new amyloid and cause regression of existing amyloid deposits.
In secondary amyloidosis, aggressive treatment of the underlying disease can improve symptoms and/or slow progression of disease. Complications such as heart failure, kidney failure, and other problems can sometimes be treated as necessary.
Occasionally, transplantation of a damaged organ is necessary. However, even after this has been carried out the new organ may become affected by amyloidosis.
Treatment may also be aimed at supporting the function of damaged tissues and treating complications such as heart or kidney failure.
Overall, many types of amyloidosis follow a steadily progressive course and may prove fatal within a year or two.
The severity of the disease depends upon the organs affected. Heart and kidney involvement may lead to organ failure and death. Systemic involvement is associated with death within 1 to 3 years.
•Congestive heart failure
•Endocrine failure (hormonal disorder)
Prevention : There is no known prevention.
Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.
- Primary amyloidosis – All Information (umm.edu)
- Multiple Myeloma and Amyloidosis (everydayhealth.com)
- Hereditary amyloidosis – All Information (umm.edu)
- Secondary systemic amyloidosis – All Information (umm.edu)
- Review of cardiac amyloidosis (doctorrw.blogspot.com)
- Former News Sentinel sports writer Nick Gates battling disease with no known cure (knoxnews.com)