In
medicine, amyloidosis refers to a variety of conditions wherein amyloid
proteins are abnormally deposited in organs or tissues and cause harm. 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 where in the body
amyloid deposits accumulate. Amyloidosis may be inherited or acquired.
AL amyloidosis can affect a wide range of organs, and
consequently present with a range of symptoms. Kidney complications are the
most commonly cited results of AL amyloidosis. Symptoms of kidney disease and
renal failure can include fluid retention, swelling, and shortness of breath. In addition to
kidneys, AL amyloidosis may affect the heart, peripheral nervous system,
gastrointestinal tract, blood, lungs and skin. Heart complications, which
affect more than a third of AL patients, include heart failure and irregular
heart beat. Other symptoms can include stroke, gastrointestinal disorders,
enlarged liver, diminished spleen function, diminished function of the adrenal
and other endocrine glands, skin color change or growths, lung problems,
bleeding and bruising problems, fatigue and weight loss
The most effective treatment is autologous bone
marrow transplants with stem cell rescues. However many patients are too weak
to tolerate this approach.
Other treatments can involve application of chemotherapy similar
to that used in multiple myeloma. A combination of bortezomib and dexamethasone has
been proposed, as has melphalan and dexamethasone.
Median
survival for patients diagnosed with AL amyloidosis is 40 months.
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