Amyloidosis is the name given to a group of rare, complicated disorders in which amyloid consisting of misfolded proteins, which are relatively insoluble, deposit and accumulate, in no particular order, in tissues and organs of the body with the exception of the brain. As these deposits progressively build up they impair the working of the organs which without treatment eventually leads to organ failure
The main types of amyloidosis, AL, ATTR, AA are referred to as systemic diseases because the amyloid can deposit in any organ of the body. Also the production of the amyloid forming protein, the precursor protein, is distant to the organs in which the amyloid forming protein is depositing.
This is different in localised amyloidosis where the amyloid-forming protein is produced and deposited in the same part of the body.
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Localised amyloidosis is also known as Localised light chain amyloidosis. Why? AL (light chain) amyloidosis is caused by an abnormal protein (the light chain of an immunoglobulin or antibody protein) made by abnormal plasma cells found in the bone marrow.
Abnormal plasma cells producing light chains are usually found in the affected tissues of patients with localised amyloidosis, however it is rarely associated with detection of a serum monoclonal protein in blood tests and has a normal free light chain ratio.
Localised amyloidosis of a non-AL type may very rarely be found incidentally in areas such as glandular tissues and blood vessels. This generally does not require treatment or follow up.
Localised AL amyloidosis is an:
- Acquired disease and is not inherited.
- The amyloid deposits are both produced and deposited in one isolated part of the body.
- The prognosis of localized amyloidosis is excellent. A study of 606 patients diagnosed with localised amyloidosis at the National Amyloidosis Centre between 1980 and 2011 concluded that only one patient progressed to systemic amyloidosis.
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