| Definition: |
A disorder in which insoluble protein fibers are deposited in tissues and organs, impairing their function.
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| Alternative Names: |
Amyloid - primary
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| Causes, incidence, and risk factors: |
The cause of primary amyloidosis is unknown, but the condition is related to abnormal production of immunoglobulins by plasma cells (a type of immune cell). Symptoms are related to the organs affected by the deposits, which can include the following: tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys. Cardiomyopathy (decreased heart function), renal failure (kidney failure), carpal tunnel syndrome (painful swelling of nerves in the wrist), malabsorption (inadequate absorption of nutrients from the intestinal tract), gastrointestinal reflux, and other conditions can result. 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.
Risk factors have not been identified. Primary amyloidosis is rare, occurring only in 1 out of 100,000 people. |
| Symptoms: |
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Additional symptoms that may be associated with this disease:
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| Signs and tests: |
A 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. This disease may also alter the results of the following tests:
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| Treatment: |
| Some patients with primary amyloidosis respond to chemotherapy directed at the abnormal plasma cells. 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 provider: |
Call 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. |
| Prevention: |
| There is no known prevention.
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