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Amyloid - primary
Primary amyloidosis is a disorder in which protein fibers are deposited in tissues and organs, harming them.
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 invade the affected organs, causing them to 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. It is related to the malignant plasma cell disorder multiple myeloma.
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.