Ten-year survivors in AL amyloidosis: characteristics and treatment pattern

Eli Muchtar, Morie A. Gertz, Martha Q. Lacy, Ronald S. Go, Francis K. Buadi, David Dingli, Martha Grogan, Omar F. AbouEzzeddine, Suzanne R. Hayman, Prashant Kapoor, Nelson Leung, Amie Fonder, Miriam Hobbs, Yi Lisa Hwa, Wilson Gonsalves, Rahma Warsame, Taxiarchis V. Kourelis, Stephen Russell, John A. Lust, Yi LinSteven Zeldenrust, Robert A. Kyle, S. Vincent Rajkumar, Shaji K. Kumar, Angela Dispenzieri

Research output: Contribution to journalArticle

6 Scopus citations

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)
Pages (from-to)588-594
Number of pages7
JournalBritish journal of haematology
Volume187
Issue number5
DOIs
StatePublished - Dec 1 2019

Keywords

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

ASJC Scopus subject areas

  • Hematology

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