1. [Current treatment of AL amyloidosis]
- Author
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Desport, Estelle, Moumas, Eric, Abraham, Julie, Delbès, Sébastien, Lacotte-Thierry, Laurence, Touchard, Guy, Fermand, Jean-Paul, Bridoux, Franck, Jaccard, Arnaud, Service de néphrologie - hémodialyse et transplantation rénale, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service d'hématologie clinique et de thérapie cellulaire [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Contrôle de la Réponse Immune B et des Lymphoproliférations (CRIBL), Centre National de la Recherche Scientifique (CNRS)-Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), Service d'Hématologie clinique et thérapie cellulaire [CHU Limoges], CHU Limoges, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Saint-Antoine [APHP], and Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS)
- Subjects
MESH: Immunoglobulin Light Chains ,Consensus Development Conferences as Topic ,Paraproteinemias ,Kaplan-Meier Estimate ,Dexamethasone ,MESH: Kidney Transplantation ,Bortezomib ,MESH: Paraproteinemias ,Natriuretic Peptide, Brain ,MESH: Renal Dialysis ,MESH: Natriuretic Peptide, Brain ,MESH: Peptide Fragments ,Lenalidomide ,Melphalan ,Randomized Controlled Trials as Topic ,MESH: Thalidomide ,MESH: Heart Transplantation ,Amyloidosis ,Prognosis ,Boronic Acids ,Thalidomide ,MESH: Kidney Failure, Chronic ,Pyrazines ,MESH: Paraproteins ,MESH: Pyrazines ,MESH: Dexamethasone ,[SDV.IMM]Life Sciences [q-bio]/Immunology ,Drug Therapy, Combination ,MESH: Boronic Acids ,Cardiomyopathies ,Amyloid ,MESH: Prognosis ,MESH: Melphalan ,Renal Dialysis ,Humans ,MESH: Amyloidosis ,MESH: Kaplan-Meier Estimate ,MESH: Amyloid ,MESH: Humans ,MESH: Consensus Development Conferences as Topic ,MESH: Biological Markers ,Kidney Transplantation ,Peptide Fragments ,MESH: Drug Therapy, Combination ,MESH: Randomized Controlled Trials as Topic ,MESH: Cardiomyopathies ,Heart Transplantation ,Kidney Failure, Chronic ,Immunoglobulin Light Chains ,Biomarkers ,Paraproteins - Abstract
International audience; 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.
- Published
- 2011
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