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Alternative namesAmyloid - primary
DefinitionPrimary amyloidosis is a disorder in which insoluble protein fibers are deposited in tissues and organs, impairing their function.
Causes, incidence, and risk factors
The cause of primary amyloidosis is unknown, but the condition is related to abnormal production of immunoglobulins by a type of immune cell called plasma cells.
The symptoms depend on the organs affected by the deposits, which can include the tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys.
This can result in the following conditions:
The deposits infiltrate the affected organs, causing them to lose resilience and become stiff, which decreases their ability to function. Secondary amyloidosis can be caused by infection, inflammatory diseases, and sometimes cancer.
Signs and testsA physical examination may show enlarged liver or spleen. There may be signs of heart failure .
If specific organ damage is suspected, testing to confirm amyloidosis of that organ may be performed. For example:
Some patients with primary amyloidosis respond to chemotherapy directed at the abnormal plasma cells. Autologous stem cell transplanation may be used, as in multiple myeloma.
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.
Expectations (prognosis)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.
Calling your health care providerCall your health care provider if symptoms consistent with primary amyloidosis develop.
If you know you have primary amyloidosis, call your health care provider if difficulty breathing, persistent swelling of the ankles or other areas, decreased urine output, or other symptoms occur. This may indicate that complications have developed.
PreventionThere is no known prevention.
Update Date: 9/14/2003Corey Cutler, M.D., M.P.H. F.R.C.P.C., Department of Medical Oncology, Dana-Farber Cancer Institute; Instructor in Medicine, Harvard University, Boston, MA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT