title: [AL amyloidosis].
abstract: AL amyloidosis belongs to the group of conformational diseases. It is the most common type of amyloidosis with an estimated 500 new cases per year in France. It is due to a small and usually indolent plasma cell clone which synthesizes an unstable, misfolded monoclonal immunoglobulin light chain that is prone to aggregate and form amyloid fibrils. Non-invasive biopsy such as abdominal fat aspiration or minor salivary gland biopsy should be performed to confirm the diagnosis and if negative, involved tissues have to be examined. Clinical presentation is very diverse, as AL amyloidosis can affect almost any organ or tissue in the body, other than the brain. The kidney is the most frequent organ involved, whereas heart disease characterized by restrictive cardiomyopathy is the most severe. Early diagnosis, before advanced cardiomyopathy, is essential for improving outcome. The association of alkylating agent and high-dose dexamethasone is effective in almost two-thirds of patients. Combinations of proteasome inhibitors, dexamethasone, and alkylating agents achieve high response rates. Close monitoring of clonal and organ response is mandatory to guide therapy changes and duration. New treatments designed to eliminate amyloid deposits are under development.
authors.affiliation: Centre national de référence de l'amylose AL et des autres maladies de dépôts d'immunoglobulines monoclonales, hôpital Dupuytren, CHU de Limoges, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France; Service d'hématologie et thérapie cellulaire, hôpital Dupuytren, CHU de Limoges, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France. Electronic address: Arnaud.Jaccard@chu-limoges.fr.
authors.forename: A
authors.initials: A
authors.lastname: Jaccard
authors.affiliation: Centre national de référence de l'amylose AL et des autres maladies de dépôts d'immunoglobulines monoclonales, hôpital Dupuytren, CHU de Limoges, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France; Service de néphrologie, hémodialyse et transplantation rénale, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France.
authors.forename: E
authors.initials: E
authors.lastname: Desport
authors.affiliation: Centre national de référence de l'amylose AL et des autres maladies de dépôts d'immunoglobulines monoclonales, hôpital Dupuytren, CHU de Limoges, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France; Service de cardiologie, hôpital Dupuytren, CHU de Limoges, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France.
authors.forename: D
authors.initials: D
authors.lastname: Mohty
authors.affiliation: Centre national de référence de l'amylose AL et des autres maladies de dépôts d'immunoglobulines monoclonales, hôpital Dupuytren, CHU de Limoges, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France; Service de néphrologie, hémodialyse et transplantation rénale, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France.
authors.forename: F
authors.initials: F
authors.lastname: Bridoux
mesh_terms: D000686:Amyloidosis / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D001706:Biopsy; D009202:Cardiomyopathies / Q000209:etiology / Q000473:pathology; D005602:France / Q000453:epidemiology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism; D000075363:Immunoglobulin Light-chain Amyloidosis; D007668:Kidney / Q000473:pathology; D011379:Prognosis; D012469:Salivary Glands / Q000473:pathology
chemical_list: D007147:Immunoglobulin Light Chains
keywords: AL amyloidosis; Amylose AL; Cardiopathie restrictive; Chaînes légères libres d’immunoglobulines; Immunoglobulin free light chains; Nephrotic syndrome; Restrictive cardiomyopathy; Syndrome néphrotique