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Primary amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid deposits.
Amyloid - primary
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.
Primary amyloidosis can lead to conditions that include:
The deposits build up in the affected organs, causing them to become stiff, which decreases their ability to function.
Risk factors have not been identified. Primary amyloidosis is rare. It is similar to multiple myeloma, and is treated the same way.
Symptoms depend on the organs affected by the deposits. These organs can include the tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys.
Other symptoms that may occur with this disease:
Your doctor may discover that you have an enlarged liver or spleen.
If organ damage is suspected, your doctor may order tests to confirm amyloidosis of that organ. For example:
This disease may also affect the results of the following tests:
Some patients with primary amyloidosis respond to chemotherapy focused on the abnormal plasma cells. A stem cell transplant may be done, as in multiple myeloma.
In secondary amyloidosis, aggressively treating the disease that is causing the excess amyloid protein can improve symptoms and/or slow the disease from getting worse. Complications such as heart failure, kidney failure, and other problems can sometimes be treated, when needed.
How severe the disease is depends on the organs that are affected. When the heart and kidney are involved, it may lead to organ failure and death. Body-wide ( systemic) involvement can lead to death in 1 to 3 years.
Call your health care provider if:
There is no known prevention for primary amyloidosis.
Buxbaum JN. The Amyloidoses. In: Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 2004.
Gertz MA, Lacy MQ, Dispenzieri A, Hayman SR. Immunoglobulin light-chain amyloidosis (primary amyloidosis). In: Hoffman R, Benz Jr. EJ, Shattil SJ, et al., eds. Hematology: Basic Principles and Practice. 5th ed. Philladelphia, Pa: Elsevier Churchill Livingston; 2008:chap 89.