Amyloidosis: Introduction

Approved by the Cancer.Net Editorial Board, 08/2016

ON THIS PAGE: You will find some basic information about this disease, which is not cancer, and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Amyloidosis. To see other pages, use the menu. Think of that menu as a roadmap to this full guide.

About amyloidosis

Amyloidosis is a rare and serious protein deposition disease. It is caused by an abnormal protein called amyloid that builds up in tissues or organs. As the amount of amyloid protein deposits increase in a tissue or organ, they interfere with the tissue or organ’s healthy function. Eventually, the amyloid protein deposits cause symptoms and organ failure. Amyloidosis is sometimes fatal.

Amyloid protein deposits, or amyloidosis, may be localized to organs, such as the lung, skin, bladder, or bowel, or they can be systemic. “Systemic” means that the deposits may be found throughout the body. Systemic amyloidosis is the most common. Although amyloidosis is not a type of cancer, it may be associated with certain blood cancers like multiple myeloma.

Amyloidosis is a very rare disorder. Because it is such a rare disorder, it has been difficult to study. However, doctors and researchers have begun to understand more about amyloidosis over the past few decades. Research continues to uncover more about this disorder.

Types of amyloidosis

There are different types of amyloidosis, including the following:

  • Light chain (AL) amyloidosis. This is the most common type of amyloidosis in the United States. The amyloid proteins that build up in the tissues in this condition are known as light chains. They can either be kappa or lambda light chains. AL amyloidosis is a disorder of the plasma cells. Plasma cells are a type of white blood cell responsible for the production of immunoglobulins or antibodies, which are a type of protein that fights infection. In AL amyloidosis, the light chain proteins are misshapen and produced in excess. They deposit in tissues and can damage 1 or more organs. The heart, kidneys, nerves, and gastrointestinal system are the most common organs affected. Because AL amyloidosis is associated with the overproduction of plasma cell proteins, it is linked to multiple myeloma.

  • Autoimmune (AA) amyloidosis. AA amyloidosis is also called “secondary amyloidosis” or “inflammatory amyloidosis.” In this condition, the amyloid protein that builds up in the tissues is called the A protein. AA amyloidosis is associated with some chronic diseases, such as diabetes, tuberculosis, rheumatoid arthritis, and inflammatory bowel disease. It may also be linked to aging. AA amyloidosis can affect the spleen, liver, kidneys, adrenal glands, and lymph nodes. Lymph nodes are tiny, bean-shaped organs that fight infection.

  • Hereditary or familial amyloidosis. Hereditary amyloidosis is rare. It can be passed from generation to generation within a family. The proteins produced in hereditary amyloidosis may cause problems with the heart and may cause carpal tunnel syndrome and eye abnormalities. The most common subtypes involve a protein called transthyretin (TTR).

This section covers AL, AA, and hereditary amyloidosis. Other types of amyloidosis include beta-2 microglobulin amyloidosis, which occurs in some patients with chronic kidney problems, and types of amyloidosis located in specific areas of the body.

The next section in this guide is Statistics. It helps explain how many people are diagnosed with this disease and general survival rates. Or, use the menu to choose another section to continue reading this guide.