Novel therapeutic options in primary systemic amyloidosis

Amyloid is defined in tissue sections as an amorphous deposit that binds the dye Congo red and demonstrates apple-green birefringence when viewed under polarized light. X-ray diffraction studies have shown that the binding of Congo red is due to the ß-pleated sheet configuration of the amyloid protein. All amyloid deposits are extracellular, and the clinical syndrome resulting from the deposition of amyloid is termed amyloidosis. This chapter discusses amyloidosis related to immunoglobulin light-chain deposits (AL amyloidosis) and current therapies directed at the management of this type of amyloidosis (1).