Ten-year survivors in AL amyloidosis

characteristics and treatment pattern

Eli Muchtar, Morie Gertz, Martha Lacy, Ronald S. Go, Francis K. Buadi, David M Dingli, Martha Grogan, Omar Abou Ezzeddine, Suzanne R. Hayman, Prashant Kapoor, Nelson Leung, Amie Fonder, Miriam Hobbs, Yi Lisa Hwa, Wilson Gonsalves, Rahma Warsame, Taxiarchis Kourelis, Stephen J Russell, John A. Lust, Yi Lin & 5 others Steven Zeldenrust, Robert A. Kyle, S Vincent Rajkumar, Shaji K Kumar, Angela Dispenzieri

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Improvement in survival in Light chain (AL) amyloidosis has been seen over recent decades, enabling more patients to achieve long-term survival. Patients with AL amyloidosis who survived ≥10 years from time of diagnosis (n = 186) were the subject of this study. Ten-year survivors represented 22% of the total population. These patients were characterized by favourable patient, organ and plasma cell features. Of note, trisomies were less common among 10-year survivors compared to those who did not survive to 10 years. All-time best haematological response was complete response in 67%, very good partial response in 30%, partial response in 2% and no response in 1%, with 11% having received a consolidative strategy for inadequate response to first line therapy. The overall organ response rate to first-line therapy was 76%, which increased to 86% when considering subsequent line(s) of therapy. Forty-seven percent of the 10-year survivors did not require a second-line therapy. The median treatment-free survival (TFS) among the 10-year survivors was 10·5 years (interquartile range 7·4-12·2). On multivariate analysis independent predictors for TFS were the achievement of complete haematological response and lack of cardiac involvement. Long-term survivors are increasingly seen in AL amyloidosis and present distinct patient, organ and clonal disease features.

Original languageEnglish (US)
JournalBritish journal of haematology
DOIs
StatePublished - Jan 1 2019

Fingerprint

Amyloidosis
Survivors
Survival
Therapeutics
Trisomy
Plasma Cells
Multivariate Analysis
Light
Population

Keywords

  • mortality
  • outcome
  • prognosis
  • response
  • treatment
  • trend

ASJC Scopus subject areas

  • Hematology

Cite this

Ten-year survivors in AL amyloidosis : characteristics and treatment pattern. / Muchtar, Eli; Gertz, Morie; Lacy, Martha; Go, Ronald S.; Buadi, Francis K.; Dingli, David M; Grogan, Martha; Abou Ezzeddine, Omar; Hayman, Suzanne R.; Kapoor, Prashant; Leung, Nelson; Fonder, Amie; Hobbs, Miriam; Hwa, Yi Lisa; Gonsalves, Wilson; Warsame, Rahma; Kourelis, Taxiarchis; Russell, Stephen J; Lust, John A.; Lin, Yi; Zeldenrust, Steven; Kyle, Robert A.; Rajkumar, S Vincent; Kumar, Shaji K; Dispenzieri, Angela.

In: British journal of haematology, 01.01.2019.

Research output: Contribution to journalArticle

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abstract = "Improvement in survival in Light chain (AL) amyloidosis has been seen over recent decades, enabling more patients to achieve long-term survival. Patients with AL amyloidosis who survived ≥10 years from time of diagnosis (n = 186) were the subject of this study. Ten-year survivors represented 22{\%} of the total population. These patients were characterized by favourable patient, organ and plasma cell features. Of note, trisomies were less common among 10-year survivors compared to those who did not survive to 10 years. All-time best haematological response was complete response in 67{\%}, very good partial response in 30{\%}, partial response in 2{\%} and no response in 1{\%}, with 11{\%} having received a consolidative strategy for inadequate response to first line therapy. The overall organ response rate to first-line therapy was 76{\%}, which increased to 86{\%} when considering subsequent line(s) of therapy. Forty-seven percent of the 10-year survivors did not require a second-line therapy. The median treatment-free survival (TFS) among the 10-year survivors was 10·5 years (interquartile range 7·4-12·2). On multivariate analysis independent predictors for TFS were the achievement of complete haematological response and lack of cardiac involvement. Long-term survivors are increasingly seen in AL amyloidosis and present distinct patient, organ and clonal disease features.",
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AU - Muchtar, Eli

AU - Gertz, Morie

AU - Lacy, Martha

AU - Go, Ronald S.

AU - Buadi, Francis K.

AU - Dingli, David M

AU - Grogan, Martha

AU - Abou Ezzeddine, Omar

AU - Hayman, Suzanne R.

AU - Kapoor, Prashant

AU - Leung, Nelson

AU - Fonder, Amie

AU - Hobbs, Miriam

AU - Hwa, Yi Lisa

AU - Gonsalves, Wilson

AU - Warsame, Rahma

AU - Kourelis, Taxiarchis

AU - Russell, Stephen J

AU - Lust, John A.

AU - Lin, Yi

AU - Zeldenrust, Steven

AU - Kyle, Robert A.

AU - Rajkumar, S Vincent

AU - Kumar, Shaji K

AU - Dispenzieri, Angela

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