title: Immunoglobulin Light Chain Systemic Amyloidosis.
abstract: Immunoglobulin light chain amyloidosis (AL) is a rare, complex disease caused by misfolded free light chains produced by a usually small, indolent plasma cell clone. Effective treatments exist that can alter the natural history, provided that they are started before irreversible organ damage has occurred. The cornerstones of the management of AL amyloidosis are early diagnosis, accurate typing, appropriate risk-adapted therapy, tight follow-up, and effective supportive treatment. The suppression of the amyloidogenic light chains using the cardiac biomarkers as guide to choose chemotherapy is still the mainstay of therapy. There are exciting possibilities ahead, including the study of oral proteasome inhibitors, antibodies directed at plasma cell clone, and finally antibodies attacking the amyloid deposits are entering the clinic, offering unprecedented opportunities for radically improving the care of this disease.
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.|Division of Laboratory Medicine, Mayo Clinic, Rochester, MN, USA.
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.affiliation: Amyloidosis Research and Treatment Center, Foundation IRCCS Policlinico San Matteo and Department of Molecular Medicine, University of Pavia, Pavia, Italy. gmerlini@unipv.it.
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
mesh_terms: D000686:Amyloidosis; D007147:Immunoglobulin Light Chains
chemical_list: D007147:Immunoglobulin Light Chains
keywords: Biomarkers; Cardiac amyloidosis; Chemotherapy; Immunoglobulin light chain amyloidosis; Immunotherapy