title: Successful sequential liver and stem cell transplantation for hepatic failure due to primary AL amyloidosis.
abstract: We report on a patient with primary AL amyloidosis who presented with progressive liver failure secondary to hepatic infiltration in the absence of significant extrahepatic involvement. Orthotopic liver transplantation was performed successfully. After an uneventful postoperative course, the patient developed evidence of significant recurrent amyloidosis requiring treatment. He then underwent stem cell transplantation 10 and 14 months after liver transplantation. After 28 months of follow-up posttransplantation, the patient continues to do well, with no clinical evidence of recurrent disease. This is the first reported patient with primary amyloidosis to undergo sequential liver and stem cell transplantation leading to resolution of the disease and only the second to undergo successful liver transplantation for this disorder.
authors.affiliation: Center for Liver Disease and Transplantation, New York-Presbyterian Hospital, New York 10036, USA.
authors.forename: K Shiva
authors.initials: KS
authors.lastname: Kumar
authors.forename: Jay
authors.initials: J
authors.lastname: Lefkowitch
authors.forename: Mark W
authors.initials: MW
authors.lastname: Russo
authors.forename: Charles
authors.initials: C
authors.lastname: Hesdorffer
authors.forename: Milan
authors.initials: M
authors.lastname: Kinkhabwala
authors.forename: Sandip
authors.initials: S
authors.lastname: Kapur
authors.forename: Jean C
authors.initials: JC
authors.lastname: Emond
authors.forename: Robert S
authors.initials: RS
authors.lastname: Brown
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000145:classification / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008099:Liver / Q000473:pathology; D017093:Liver Failure / Q000209:etiology* / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D016896:Treatment Outcome