Definition:
Primary amyloidosis is a disorder in which protein fibers are deposited in tissues and organs, harming them.
Alternative Names:
Amyloid - primary
Causes, incidence, and risk factors:
The cause of primary amyloidosis is unknown, but the condition is related to abnormal production of antibodies by a type of immune cell called plasma cells. The 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. Primary amyloidosis can result in 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.
References:
Buxbaum JN. The Amyloidoses. In: Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 2004. Hoffman R, Benz Jr. EJ, Shattil SJ, et al., eds. Hematology: Basic Principles and Practice. 4th ed. Philladelphia, Pa: Churchill Livingston; 2005:1540-48.
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