Amyloidosis

Types

Primary (AL)

The most common form of amyloidosis is a disease of the bone marrow called primary systemic amyloidosis (immunoglobulin light chain or AL). Normally, bone marrow cells — called plasma cells — make antibody proteins that protect against infection and disease. In healthy individuals, the body uses and recycles these antibodies. In AL amyloidosis, excess antibody proteins are produced and recycling does not occur properly. These proteins can stick together in body tissues, building the substance called amyloid. Symptoms vary, depending on the organ(s) affected. Amyloidosis can be disabling or life threatening.

AL amyloidosis affects about eight persons per million annually. Its cause is unknown, and anyone can be affected. Most people who develop amyloidosis are over 40 years old; 63 is the average age at diagnosis. Two-thirds of patients with primary amyloidosis are men.

AL amyloidosis is neither contagious nor inherited. But, as shown in this table,other forms of amyloidosis are inherited. About 10 percent of patients who have multiple myeloma (a form of bone marrow cancer) develop AL amyloidosis.

Secondary

Secondary amyloidosis usually occurs in tandem with chronic infectious or inflammatory diseases, such as rheumatoid arthritis, tuberculosis or osteomyelitis (a bone infection). Secondary amyloidosis generally affects a patient's kidneys, spleen, liver and lymph nodes, though other organs may be involved. Treatment of the underlying disease may also help the secondary amyloidosis.

Hereditary

Hereditary amyloidosis, the inherited form, typically affects the nerves, kidneys and heart.

View a more detailed breakdown of the types of amyloidosis (PDF).