Karlijn H G Rutten1, Reinier A P Raymakers1, Bouke P C Hazenberg2, Hans L A Nienhuis2, Edo Vellenga3, Monique C Minnema1. 1. a Department of Haematology , Utrecht University, University Medical Centre Utrecht , Utrecht , The Netherlands. 2. b Department of Rheumatology & Clinical Immunology , University of Groningen, University Medical Centre Groningen , Groningen , The Netherlands. 3. c Department of Haematology , University of Groningen, University Medical Centre Groningen , Groningen , The Netherlands.
Abstract
BACKGROUND: Although survival has improved in recent decades, the short-term prognosis of patients with immunoglobulin light chain (AL) amyloidosis remains grim. We aimed to assess overall survival (OS) of AL amyloidosis patients by comparing cohorts in two consecutive time periods. METHODS: Data were collected and compared on 126 patients from two tertiary referral centres in The Netherlands during the time periods 2008-2012 and 2013-2016. RESULTS: There was a non-significant trend to improved 6-month OS in the last cohort (78% vs. 67%, p = .216, crude odds ratio 1.66, 95%CI 0.74-3.70, adjusted odds ratio 2.22, 95%CI 0.88-5.56). Patients in this cohort had higher Mayo risk scores (stage III 40% vs. 24%, p < .001 and revised stage IV 14% vs. 11%, p < .001), higher use of bortezomib (50% vs. 30%), and better haematological response (complete response/very good partial response in 39% vs. 27%, p < .001). Diagnostic delay was similar in both time periods. CONCLUSIONS: In the 2013-2016 cohort there was a trend toward improved 6-month OS, and an improved haematological response. Patients in this cohort had more advanced cardiac disease and received bortezomib more frequently, but diagnostic delay was similar to the 2008-2012 cohort. For further prognostic improvement, practitioners should be more alert, especially for cardiac amyloidosis.
BACKGROUND: Although survival has improved in recent decades, the short-term prognosis of patients with immunoglobulin light chain (AL) amyloidosis remains grim. We aimed to assess overall survival (OS) of AL amyloidosispatients by comparing cohorts in two consecutive time periods. METHODS: Data were collected and compared on 126 patients from two tertiary referral centres in The Netherlands during the time periods 2008-2012 and 2013-2016. RESULTS: There was a non-significant trend to improved 6-month OS in the last cohort (78% vs. 67%, p = .216, crude odds ratio 1.66, 95%CI 0.74-3.70, adjusted odds ratio 2.22, 95%CI 0.88-5.56). Patients in this cohort had higher Mayo risk scores (stage III 40% vs. 24%, p < .001 and revised stage IV 14% vs. 11%, p < .001), higher use of bortezomib (50% vs. 30%), and better haematological response (complete response/very good partial response in 39% vs. 27%, p < .001). Diagnostic delay was similar in both time periods. CONCLUSIONS: In the 2013-2016 cohort there was a trend toward improved 6-month OS, and an improved haematological response. Patients in this cohort had more advanced cardiac disease and received bortezomib more frequently, but diagnostic delay was similar to the 2008-2012 cohort. For further prognostic improvement, practitioners should be more alert, especially for cardiac amyloidosis.
Entities:
Keywords:
AL amyloidosis; prognosis; survival; time trend
Authors: M I F J Oerlemans; K H G Rutten; M C Minnema; R A P Raymakers; F W Asselbergs; N de Jonge Journal: Neth Heart J Date: 2019-11 Impact factor: 2.380
Authors: Maaike Brons; Steven A Muller; Frans H Rutten; Manon G van der Meer; Alexander F J E Vrancken; Monique C Minnema; Annette F Baas; Folkert W Asselbergs; Marish I F J Oerlemans Journal: Eur Heart J Open Date: 2022-02-24