title: Immunoglobulin light chain amyloidosis.
abstract: Primary light chain amyloidosis is the most common form of systemic amyloidosis and is caused by misfolded light chains that cause proteotoxicity and rapid decline of vital organ function. Early diagnosis is essential in order to deliver effective therapy and prevent irreversible organ damage. Accurate diagnosis requires clinical skills and advanced technologies. The disease can be halted and the function of target organs preserved by the prompt reduction and elimination of the plasma cell clone producing the toxic light chains in the bone marrow. Heart damage is the major determinant of survival, and staging with cardiac biomarkers guides treatment. Two-thirds of patients can benefit from treatment with improved quality of life and extended survival. Future efforts should be directed at early diagnosis, improving the tolerability and efficacy of anti-plasma cell therapy, accelerating recovery of organ function via promoting resorption of amyloid deposits, and developing novel approaches to counter light chain proteotoxicity.
authors.affiliation: Department of Molecular Medicine, University of Pavia, Foundation Scientific Institute San Matteo, Amyloidosis Research and Treatment Center, V.le Golgi 19 27100, Pavia, Italy.
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
authors.forename: Raymond L
authors.initials: RL
authors.lastname: Comenzo
authors.forename: David C
authors.initials: DC
authors.lastname: Seldin
authors.forename: Ashutosh
authors.initials: A
authors.lastname: Wechalekar
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
mesh_terms: D000686:Amyloidosis / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D004232:Diuretics / Q000627:therapeutic use; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007136:Immunoglobulins / Q000097:blood / Q000652:urine; D007155:Immunologic Factors / Q000627:therapeutic use; D016377:Organ Transplantation; D034622:RNA Interference; D033581:Stem Cell Transplantation
chemical_list: D004232:Diuretics; D007147:Immunoglobulin Light Chains; D007136:Immunoglobulins; D007155:Immunologic Factors; C009823:M-proteins (Myeloma)