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Types of Amyloidosis

Amyloidisis is a disorder caused by an abnormal build up of the protein called amyloid, which is produced by cells in the bone marrow. An abnormal amyloid buildup can affect tissues and different organs, including the nervous system, digestive tract, heart, liver and spleen. The three main types of systematic amyloidosis are primary amyloidosis, secondary amyloidosis and familial amyloidosis. Other types of amyloidosis are categorized according to the organ they affect.

  1. Primary Amyloidosis

    • Primary amyloidosis (AL) is the most common form of amyloidosis in the United States. According to the Amyloidosis Foundation, approximately 2000 new cases of primary amyloidosis are diagnosed in the United States every year. Primary amyloidosis is caused by buildup of the amyloid light (AL) chain proteins. Primary amyloidosis is a condition that occurs by itself. In rare cases, it can also occur along with multiple myeloma. The common symptoms of primary amyloidosis include breathlessness, edema, diarrhea, weight loss and swollen tongue. Treatments for primary amyloidosis include chemotherapy and bone marrow transplantation.

    Secondary Amyloidosis

    • Secondary amyloidosis, also known as amyloid amyloidosis (AA), occurs as a complication of inflammatory or infectious diseases, such as, rheumatoid arthritis, granulomatous ileitis or osteomyelitis. Primary amyloidosis is caused by buildup of the AA protein. Treating the underlying condition with anti-inflammatory medication can help diminish amyloid buildup in the tissues and organs. A new treatment method, which inhibits the production of the AA protein, is under development.

    Familial Amyloidosis

    • Familial (ATTR) amyloidosis is the only hereditary type of amyloidosis out of the 21 different types. Familial amyloidosis is caused by a mutation in the chromosome 5 gene, which results in the build up of the transthyretin (TTR) protein. Researchers believe that since the liver produces the TTR protein, a liver transplant may stop the production of the TTR protein. The chance that the children of a person with familial amyloidosis will inherit the defected gene is about 50 percent.

    B-2 Microglobulin Amyloidosis

    • People who suffer from kidney failure, or have been on long-term dialysis, may develop b-2 microglobulin amyloidosis. The b-2 microglobulin protein builds up in the tissues around the joints because the kidney is unable to remove the b-2 microglobulin protein from the body.

    Localized Amyloidosis

    • Primary, secondary and familial amyloidosis are all forms of systemic amyloidosis, which affects the body as a whole. There are also different types of localized amyloidosis, which affects a specific part of the body. The most common type of localized amyloidosis is Alzheimer's disease, which is caused by build of the b-amyloid protein in the brain. The amyloid protein that causes localized amyloid is not produced in the bone marrow, but in the tissues.

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