Primary amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid deposits.
Alternative Names
Amyloid - primary
Causes, incidence, and risk factors
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:
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.
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:
This condition is treated the same way as multiple myeloma.
Treatment may include:
Chemotherapy
Stem cell transplant
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.
Expectations (prognosis)
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.
Review Date:
1/1/2013
Reviewed 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.
