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Amyloidosis
Condition Overview: Amyloidosis is a group of disorders caused by abnormal folding of proteins leading to fibril formation in one or more body organs, systems or soft tissues. These clumps of protein are called amyloid deposits and the accumulation of amyloid deposits causes the progressive malfunction and eventual failure of the affected organ. Normally, proteins are broken down at about the same rate as they are produced, but these unusually stable amyloid deposits are deposited more rapidly than they can be broken down. The accumulation may be localized in one organ or may be systemic such that several organs are affected.The different types of systemic amyloidosis are sometimes classified as primary, secondary or hereditary. Primary amyloidosis (also called AL) is the most common type of systemic amyloidosis. It is caused by an abnormal plasma cell in the bone marrow and sometimes occurs with multiple myeloma. Secondary amyloidosis (also called AA) occurs in association with another disease such as rheumatoid arthritis, familial Mediterranean fever or other chronic infection or inflammatory disease. Hereditary amyloidosis is a rare type of amyloidosis that is caused by an abnormal gene. There are several abnormal genes that can cause hereditary amyloidosis, but the most common type of hereditary amyloidosis is called ATTR and caused by a mutations in the TTR gene. Beta2-microglobulin amyloidosis (Aß2M) is a type of systemic amyloidosis that can occur in patients who have experienced long-term kidney dialysis to remove accumulated impurities or wastes in the blood by mechanical filtration. . |
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