Early intensification and autologous stem cell transplantation in patients with systemic AL amyloidosis: a single-centre experience.
Details
State: Public
Version: Final published version
License: Not specified
It was possible to publish this article open access thanks to a Swiss National Licence with the publisher.
Serval ID
serval:BIB_D7AFA2AA6B11
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Early intensification and autologous stem cell transplantation in patients with systemic AL amyloidosis: a single-centre experience.
Journal
Annals of Hematology
ISSN
1432-0584 (Electronic)
ISSN-L
0939-5555
Publication state
Published
Issued date
2009
Volume
88
Number
7
Pages
681-685
Language
english
Notes
Publication types: Clinical Trial ; Journal Article
Publication Status: ppublish
Publication Status: ppublish
Abstract
Primary systemic amyloidosis (AL amyloidosis) continues to have a very poor prognosis. Most therapeutic strategies remain unsatisfactory. Conventional chemotherapy is known to offer at best only moderate efficacy. Several studies have yielded higher complete response rates after high-dose chemotherapy and autologous stem cell transplantation (ASCT) in addition to improving outcomes in a subgroup of patients. However, the superiority of an intensive approach in AL amyloidosis has not been confirmed in a randomised trial. The precise role of ASCT remains unclear. We report our experience in 16 patients diagnosed with AL amyloidosis and treated in a multidisciplinary approach with high-dose melphalan and ASCT. Median age was 59 (39-71) years. The kidneys were predominantly affected in 75% of cases; two or more organs were affected in 38%. Median time from diagnosis to transplantation was 2 (1-4) months. Three patients (19%) developed acute renal failure and required transient dialysis. Transplant-related mortality was 6% after 100 days. Haematological complete response (CR) was obtained in nine (56%) and organ response in six (38%) patients. Nine out of 12 patients (75%) with kidney involvement exhibited a sustained clinical benefit at 12 months. Half of all the patients (n = 8) were alive after a median follow-up of 33 months, including two in continuous CR. This suggests that high-dose chemotherapy and ASCT are still valid treatment options in AL amyloidosis and that a significant number of patients with renal involvement might benefit from this approach.
Keywords
Adult, Aged, Amyloidosis/complications, Amyloidosis/mortality, Female, Hematopoietic Stem Cell Transplantation/adverse effects, Hematopoietic Stem Cell Transplantation/methods, Humans, Kidney Diseases/therapy, Male, Melphalan/administration & dosage, Middle Aged, Myeloablative Agonists/administration & dosage, Remission Induction, Survival Rate, Transplantation, Autologous, Treatment Outcome
Pubmed
Web of science
Open Access
Yes
Create date
04/03/2009 17:24
Last modification date
14/02/2022 8:57