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Primary amyloidosis

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Primary amyloidosis

Definition:

Primary amyloidosis is a disorder in which insoluble protein fibers are deposited in tissues and organs, impairing their function.

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 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:

• Cardiomyopathy (poor heart function)
• Kidney failure
• Carpal tunnel syndrome (painful swelling of nerves in the wrist)
• Malabsorption (inadequate absorption of nutrients from the intestinal tract)
• Gastrointestinal reflux (GERD)
• Other 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.

Risk factors have not been identified. Primary amyloidosis is rare. It is related to the malignant plasma cell disorder multiple myeloma.

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