Amyloidosis: Recognition, confirmation, prognosis, and therapy

Amyloidosis should be considered in any patient older than 40 years who has nephrotic syndrome, congestive heart failure (not on an ischemic basis), i diopathic peripheral neuropathy, or unexplained hepatomegaly, When a patien t has one of these problems, immunoelectrophoresis and immunofixation of th e serum and urine should be done for the detection of a monoclonal light ch ain. If a monoclonal light chain is found, a diagnosis usually can be estab lished by amyloid stains performed on a bone marrow biopsy specimen or a su bcutaneous fat aspirate. The presence or absence of cardiac involvement wit h. amyloid is the most important prognostic factor, Treatment can range fro m observation to oral chemotherapy to hematopoietic stem cell transplantati on. A practical understanding of the mechanisms underlying this disease can lead to prompt diagnosis and early therapeutic intervention.