Amyloidosis

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Amyloidosis is a group of diseases in which one or more organ systems in the body accumulate deposits of abnormal proteins.  Although amyloidosis is not cancer, it is very serious. It is disabling or life threatening. However, growing awareness of the condition seems to be leading to substantial new research and treatment alternatives.

These are the major types of amyloidosis which are all very different from each other:

Primary Amyloidosis is a plasma cell disorder that originates in the bone marrow and is usually treated with chemotherapy. It is the most common type of amyloidosis in the United States, with estimates of up to 2000 cases diagnosed each year, and occasionally occurs with multiple myeloma. The deposits in this type of the disease are made up of immunoglobulin light chain proteins that may be deposited in any bodily tissues or organs. The disease results when enough amyloid protein builds up in one or more organs to cause the organ(s) to malfunction. The heart, kidneys, nervous system and gastrointestinal tract are most often affected.

Normally, bone marrow makes protective antibodies, which are proteins that protect against infection and disease. After they have served their function, these antibodies are broken down and recycled in the body. With amyloidosis, cells in the bone marrow produce antibodies that cannot be broken down. These antibodies then begin to build up in the bloodstream. Ultimately, they leave the bloodstream and can deposit in the tissues or organs as amyloid.

Secondary Amyloidosis is caused by a chronic infection or inflammatory disease such as rheumatoid arthritis, familial Mediterranean fever, osteomyelitis, or granulomatous ileitis. The deposits in this type of the disease are made up of a protein called the AA protein. Medical or surgical treatment of the underlying chronic infection or inflammatory disease can slow or stop the progression of this type of amyloid.

Familial (or Hereditary) Amyloidosis is the only type of amyloidosis that is inherited. It is a rare form of the disease that is found in families of nearly every ethnic background. The deposits in this type are most commonly made up of the transthyretin protein that is manufactured in the liver. It is a mutation of such a protein that causes this form of amyloidosis.

Other Types of Amyloidosis include localized amyloid, b2 micro globulin amyloid, and Alzheimer's disease. Localized types of amyloidosis are associated with hormone proteins, aging, or specific areas of the body, and have not been found to have systemic implications. The type of amyloidosis that is due to the b2 micro globulin protein may affect people who have been on dialysis for a significant length of time. In Alzheimer's disease, the amyloid protein in the brain is called the b-amyloid protein.