Abstract
The survival of patients achieving a cardiac complete response in light chain amyloidosis, defined as N-terminal pro B-type natriuretic peptide ≤350 pg/mL or B-type natriuretic peptide ≤80 pg/mL, was similar to that of a matched general population, with estimated 5-year survival rates of 93% and 95%, respectively.
References
1.
Merlini
G
, Dispenzieri
A
, Sanchorawala
V
, et al. Systemic immunoglobulin light chain amyloidosis
. Nat Rev Dis Primers
. 2018
;4
(1
):38
.2.
Dispenzieri
A
, Gertz
MA
, Kyle
RA
, et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis
. J Clin Oncol
. 2004
;22
(18
):3751
-3757
.3.
Muchtar
E
, Dispenzieri
A
, Gertz
MA
, et al. Treatment of AL amyloidosis: Mayo Stratification of Myeloma and Risk-Adapted Therapy (mSMART) consensus statement 2020 update
. Mayo Clin Proc
. 2021
;96
(6
):1546
-1577
.4.
Gertz
MA
, Cohen
AD
, Comenzo
RL
, et al. Birtamimab plus standard of care in light-chain amyloidosis: the phase 3 randomized placebo-controlled VITAL trial
. Blood
. 2023
;142
(14
):1208
-1218
.5.
Palladini
G
, Dispenzieri
A
, Gertz
MA
, et al. New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes
. J Clin Oncol
. 2012
;30
(36
):4541
-4549
.6.
Kaufman
GP
, Dispenzieri
A
, Gertz
MA
, et al. Kinetics of organ response and survival following normalization of the serum free light chain ratio in AL amyloidosis
. Am J Hematol
. 2015
;90
(3
):181
-186
.7.
Manwani
R
, Cohen
O
, Sharpley
F
, et al. A prospective observational study of 915 patients with systemic AL amyloidosis treated with upfront bortezomib
. Blood
. 2019
;134
(25
):2271
-2280
.8.
Milani
P
, Basset
M
, Nuvolone
M
, et al. Indicators of profound hematologic response in AL amyloidosis: complete response remains the goal of therapy
. Blood Cancer J
. 2020
;10
(8
):90
.9.
Muchtar
E
, Dispenzieri
A
, Leung
N
, et al. Depth of organ response in AL amyloidosis is associated with improved survival: grading the organ response criteria
. Leukemia
. 2018
;32
(10
):2240
-2249
.10.
Lilleness
B
, Ruberg
FL
, Mussinelli
R
, Doros
G
, Sanchorawala
V
. Development and validation of a survival staging system incorporating BNP in patients with light chain amyloidosis
. Blood
. 2019
;133
(3
):215
-223
.11.
Muchtar
E
, Dispenzieri
A
, Wisniowski
B
, et al. Graded cardiac response criteria for patients with systemic light chain amyloidosis
. J Clin Oncol
. 2023
;41
(7
):1393
-1403
.12.
Perme
MP
, Pavlic
K
. Nonparametric relative survival analysis with the R package relsurv
. J Stat Softw
. 2018
;87
(8
):1
-27
.13.
Therneau
TM
, Lumley
T
, Atkinson
E
, Crowson
C
. A package for survival analysis in R. R package version 3.5-7
. 2023. Accessed 28 April 2024. https://CRAN.R-project.org/package=survival.14.
Team RC R: A language and environment for statistical computing URL
. R Foundation for Statistical Computing. 2021
Accessed 28 April 2024. https://www.R-project.org/.15.
Muchtar
E
, Wisniowski
B
, Palladini
G
, et al. Graded renal response criteria for light chain (AL) amyloidosis [abstract]
. Blood
. 2021
;138
(suppl 1
):2721
.© 2024 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.
2024
You do not currently have access to this content.
This feature is available to Subscribers Only
Sign In or Create an Account Close Modal