ABSTRACT

Transthyretin (TTR) is the major component of amyloid fibrils in familial TTR amyloidosis and senile cardiac amyloidosis. In familial TTR amyloidosis, the fibrils contain a mutant TTR. The disease is usually characterized by peripheral and autonomic polyneuropathy, but the heart and other organs may be affected. In senile cardiac amyloidosis, the fibrils contain normal TTR. Deposits are present predominantly in the heart and blood vessel walls, with approximately 25 percent of the population over 80 years of age affected. The chapter aims to report a case of senile cardiac amyloidosis where an immunoglobulin lambda light chain was codeposited with normal TTR in the amyloid. Analysis of the cardiac amyloid showed that while the major protein component in the fibrils was normal TTR, a monoclonal immunoglobulin lambda light chain was also codeposited as a minor constituent in this case of senile cardiac amyloid.