title: [Current treatment of AL amyloidosis].
abstract: Systemic AL amyloidosis is a rare complication of monoclonal gammopathies. Renal manifestations are frequent, mostly characterized by heavy proteinuria, with nephrotic syndrome and renal failure in more than half of the patients at diagnosis. Without treatment, median survival does not exceed 12 months. Amyloid heart disease and diffusion of amyloid deposits are associated with reduced survival. Treatment of systemic AL amyloidosis has been profoundly modified with the introduction of international criteria for the definition of organ involvement and hematologic response, and with the use of sensitive tests for the measurement of serum-free light chain levels. Melphalan plus dexamethasone is now established as the gold standard for first line treatment of systemic AL, with similar efficacy and reduced treatment-related mortality compared to high-dose therapy. Modern chemotherapy regimens, based on the use of novel agents such as bortezomib and lenalidomide, might further improve patient survival.
authors.affiliation: Service de néphrologie et transplantation rénale, hôpital Jean-Bernard, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France.
authors.forename: Estelle
authors.initials: E
authors.lastname: Desport
authors.forename: Eric
authors.initials: E
authors.lastname: Moumas
authors.forename: Julie
authors.initials: J
authors.lastname: Abraham
authors.forename: Sébastien
authors.initials: S
authors.lastname: Delbès
authors.forename: Laurence
authors.initials: L
authors.lastname: Lacotte-Thierry
authors.forename: Guy
authors.initials: G
authors.lastname: Touchard
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Fermand
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
authors.forename: Arnaud
authors.initials: A
authors.lastname: Jaccard
mesh_terms: D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000378:metabolism; D015415:Biomarkers; D001897:Boronic Acids / Q000627:therapeutic use; D000069286:Bortezomib; D009202:Cardiomyopathies / Q000209:etiology / Q000601:surgery; D003245:Consensus Statements as Topic; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D016027:Heart Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism; D053208:Kaplan-Meier Estimate; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000601:surgery / Q000628:therapy; D016030:Kidney Transplantation; D000077269:Lenalidomide; D008558:Melphalan / Q000008:administration & dosage / Q000627:therapeutic use; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010265:Paraproteinemias / Q000150:complications / Q000188:drug therapy; D010266:Paraproteins / Q000378:metabolism; D010446:Peptide Fragments / Q000097:blood; D011379:Prognosis; D011719:Pyrazines / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic; D006435:Renal Dialysis; D013792:Thalidomide / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D000682:Amyloid; D015415:Biomarkers; D001897:Boronic Acids; D007147:Immunoglobulin Light Chains; D010266:Paraproteins; D010446:Peptide Fragments; D011719:Pyrazines; C035222:amyloid protein AL; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D013792:Thalidomide; D000069286:Bortezomib; D003907:Dexamethasone; D000077269:Lenalidomide; D008558:Melphalan