ABSTRACT

Monoclonal immunoglobulin light chains are responsible for the characteristic pathologic features found in patients with light chain-related diseases that include Multiple Myeloma and Light Chain amyloidosis (AL). This chapter presents different light chains from cardiac amyloid patients with survival ranging from 6 months to 10 years. Amyloid fibrils and/or their precursors are suggested to lead to cell death and tissue degeneration. The most aggressive AL protein is partially unfolded and with exposed hydrophobic patches shown by binding to 8-Anilino-1-naphthalenesulfonic acid. Amyloidoses are an important group of protein deposition disorders in which normally soluble protein aggregates to form insoluble extracellular amyloid fibrils. AL is characterized by the deposition of immunoglobulin light chain in multiple vital organs, where the deposits cause organ failure leading to death.