title: Successful heart transplantation following melphalan plus dexamethasone therapy in systemic AL amyloidosis.
abstract: Recurrence in the allograft and progression in other organs increase mortality after cardiac transplantation in AL amyloidosis. Survival may be improved after suppression of monoclonal light chain (LC) production following high dose melphalan and autologous stem cell transplantation (HDM/ASCT). However, because of high treatment related mortality, this tandem approach is restricted to few patients without significant extra-cardiac involvement. A diagnosis of systemic AL amyloidosis was established in a 45-year old patient with congestive heart failure related to restrictive cardiomyopathy, nephrotic syndrome, peripheral neuropathy, postural hypotension, macroglossia, and lambda LC monoclonal gammopathy. After melphalan and dexamethasone (M-Dex) therapy, which resulted in 80% reduction of serum free lambda LC, he underwent orthotopic cardiac transplantation. Two years later, he remains in a sustained hematologic remission, with no evidence of allograft or extra-cardiac amyloid accumulation. M-Dex should be considered as an alternative therapy in AL amyloid heart transplant recipients ineligible for HDM/ASCT.
authors.affiliation: Department of Clinical Hematology, CHU Limoges, 1 avenue Martin Luther King, 87000 Limoges, France. arnaud.jaccard@chu-limoges.fr
authors.forename: Aude
authors.initials: A
authors.lastname: Mignot
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
authors.forename: Antoine
authors.initials: A
authors.lastname: Thierry
authors.forename: Shaida
authors.initials: S
authors.lastname: Varnous
authors.forename: Myriam
authors.initials: M
authors.lastname: Pujo
authors.forename: Annick
authors.initials: A
authors.lastname: Delcourt
authors.forename: Jean Marc
authors.initials: JM
authors.lastname: Gombert
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Goujon
authors.forename: Fréderic
authors.initials: F
authors.lastname: Favreau
authors.forename: Guy
authors.initials: G
authors.lastname: Touchard
authors.forename: Daniel
authors.initials: D
authors.lastname: Herpin
authors.forename: Arnaud
authors.initials: A
authors.lastname: Jaccard
mesh_terms: D000686:Amyloidosis / Q000209:etiology / Q000601:surgery*; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D006084:Graft Rejection / Q000517:prevention & control; D006333:Heart Failure / Q000209:etiology / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D007146:Immunoglobulin lambda-Chains / Q000032:analysis; D008297:Male; D008558:Melphalan / Q000008:administration & dosage / Q000627:therapeutic use*; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000209:etiology; D010265:Paraproteinemias / Q000150:complications*; D010523:Peripheral Nervous System Diseases / Q000209:etiology; D055502:Secondary Prevention
chemical_list: D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains; D003907:Dexamethasone; D008558:Melphalan