Primary amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid deposits.
The cause of primary amyloidosis is unknown. The condition is related to abnormal and excess production of antibodies by a type of immune cell called plasma cells. Clumbs of abnormal proteins build up in certain organs. This reduces their ability to work correctly.
Primary amyloidosis can lead to conditions that include:
Primary amyloidosis is rare. It is similar to multiple myeloma.
Symptoms depend on the organs affected. This disease can affect the tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys.
Other symptoms that may occur with this disease:
The doctor or nurse will examine you. You will be asked questions about your medical history and symptoms. A physical exam may show that you have an swollen liver or spleen.
The first step in diagnosing amyloidosis should be blood and urine tests to look for abnormal proteins.
Other tests depend on your symptoms and what organ may be affected. Some tests include:
Tests that can help confirm the diagnosis include:
This disease may also affect the results of the following tests:
This condition is treated the same way as multiple myeloma.
Treatment may include:
If the condition is caused by another disease, that disease should be aggressively treated. This may improve symptoms or slow the disease from getting worse. Complications such as heart failure, kidney failure, and other problems can sometimes be treated, when needed.
How well you do depends on which organs are affected. Heart and kidney involvement may lead to organ failure and death. Body-wide ( systemic) amyloidosis can lead to death in 1 to 3 years.
Call your health care provider if you have symptoms of this disease. Also call if you have been diagnosed with this disease and have:
There is no known prevention for primary amyloidosis.
Amyloid - primary
Gertz MA. Amyloidosis. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 194.
Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, David R. Eltz, Stephanie Slon, and Nissi Wang.
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