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Understanding real-world treatment patterns and clinical outcomes in AL amyloidosis patients diagnosed in Canada: A population-based cohort study.


ABSTRACT: Amyloid light chain (AL) amyloidosis is a rare and chronic bone marrow disorder. Existing claims data can be used to help understand the real-world treatment patterns and outcomes of this patient population. Various population-based administrative databases in Alberta, Canada were queried from 2010 to mid-2019 to identify cases of AL amyloidosis. Baseline patient and disease characteristics, sequencing of pharmacologic therapies, overall survival, and healthcare resource utilization were evaluated. A total of 215 individuals with AL amyloidosis were included. Among patients diagnosed between 2012 and 2019, 149 (85.1%) initiated first-line, 67 (38.3%) initiated second-line, 22 (12.6%) initiated third-line, and 11 (6.3%) initiated fourth-line systemic therapy. In the first-line setting, 99/149 (66.4%) received bortezomib, cyclophosphamide, and dexamethasone (CyBorD) and 21/149 (14.1%) received another bortezomib-based regimen. Survival from time of diagnosis improved over time, with a median overall survival of 25.8 months (95% CI: 9.8, 57.1) for individuals diagnosed in 2010-2011 versus 52.1 months (95% CI: 25.6, NA) for those diagnosed in 2012-2019. Despite this improvement, the proportion of individuals diagnosed in 2012-2019 who survived beyond five-years remained low (5-year survival: 48.4%; 95% CI: 40.9, 57.2) which highlights an unmet need for more efficacious therapies.

SUBMITTER: Jimenez-Zepeda VH 

PROVIDER: S-EPMC9713227 | biostudies-literature | 2022 Nov

REPOSITORIES: biostudies-literature

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Understanding real-world treatment patterns and clinical outcomes in AL amyloidosis patients diagnosed in Canada: A population-based cohort study.

Jimenez-Zepeda Victor H VH   Reece Donna D   Rigo Rodrigo R   Gogna Priyanka P   Kong Shiying S   Hu Xun Yang XY   Chapani Parv P   Cheung Winson Y WY   Brenner Darren R DR   Plante Richard R   Shi Kun K   Husain Asad A   Tankala Dipti D   Boyne Devon J DJ  

EJHaem 20220905 4


Amyloid light chain (AL) amyloidosis is a rare and chronic bone marrow disorder. Existing claims data can be used to help understand the real-world treatment patterns and outcomes of this patient population. Various population-based administrative databases in Alberta, Canada were queried from 2010 to mid-2019 to identify cases of AL amyloidosis. Baseline patient and disease characteristics, sequencing of pharmacologic therapies, overall survival, and healthcare resource utilization were evaluat  ...[more]

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