title: Successful long-term outcome of the first combined heart and kidney transplant in a patient with systemic Al amyloidosis.
abstract: Simultaneous cardiac and renal involvement is associated with a particularly poor prognosis in patients with AL amyloidosis (AL-A). We report the first case of a successful long-term outcome of combined heart and kidney transplantation not followed by autologous stem cell transplantation in a patient with systemic AL-A. The recipient was a 46-year-old man with end-stage renal failure associated with serious cardiac involvement in the context of AL-A. Before transplantation, two courses of oral melphalan plus prednisone induced partial hematologic remission, as shown by the decrease in circulating free light chain with no improvement of renal or heart function. The patient underwent combined heart and kidney transplantation as a rescue treatment. During the follow-up period (36 months), plasma cell dyscrasia remains in complete remission, with normal free lambda light chain levels and no recurrence of amyloid deposition on heart and kidney grafts. This case report demonstrates that combined heart and kidney transplantation not systematically associated with stem cell transplantation may be considered an additional therapeutic option in AL-A patients with severe organ dysfunction and partial hematologic remission.
authors.affiliation: APHP, Hôpital Henri Mondor, Department of Nephrology and Renal Transplantation; Institut Francilien de recherche en Néphrologie et Transplantation (IFRNT), Univ Paris 12, Créteil, 94010, France.
authors.forename: V
authors.initials: V
authors.lastname: Audard
authors.forename: M
authors.initials: M
authors.lastname: Matignon
authors.forename: L
authors.initials: L
authors.lastname: Weiss
authors.forename: P
authors.initials: P
authors.lastname: Remy
authors.forename: A
authors.initials: A
authors.lastname: Pardon
authors.forename: C
authors.initials: C
authors.lastname: Haioun
authors.forename: K
authors.initials: K
authors.lastname: Belhadj
authors.forename: L
authors.initials: L
authors.lastname: Salomon
authors.forename: M L
authors.initials: ML
authors.lastname: Hillon
authors.forename: D
authors.initials: D
authors.lastname: Sahali
authors.forename: E
authors.initials: E
authors.lastname: Vermes
authors.forename: P
authors.initials: P
authors.lastname: Lang
authors.forename: P
authors.initials: P
authors.lastname: Grimbert
mesh_terms: D000686:Amyloidosis / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D016030:Kidney Transplantation; D008297:Male; D008558:Melphalan / Q000008:administration & dosage; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D019172:Transplantation Conditioning; D016896:Treatment Outcome
chemical_list: D008558:Melphalan; D011241:Prednisone