Benefit of daratumumab-based treatment in advanced cardiac stage light chain amyloidosis

Abstract Background Advanced cardiac-stage light chain (AL) amyloidosis has a poor prognosis. Other than in early-stage disease, Daratumumab’s role is less well established. Methods In this multi-center, retrospective study we analyzed patients with newly diagnosed AL amyloidosis with cardiac involvement and baseline NT-proBNP of ≥ 1800 ng/l, enrolled in the Swiss Amyloidosis Registry. We compared overall survival (OS), progression-free survival 1 and 2 (PFS), and treatment response in patients receiving Daratumumab as first- or second-line therapy to those who never received Daratumumab. Results Seventy six patients were included, median baseline NT-proBNP was 4’180 ng/l. 25 and 51 patients had 2012 revised Mayo stage III and IV disease. Patients treated with Daratumumab showed significantly higher OS and improved PFS2 compared with those who never received Daratumumab (2y-OS 75.8% vs. 52.2%, log-rank < 0.05, 2y-PFS2 75.4% vs. 58.4%, log-rank: 0.068). No significant difference was observed between patients treated with versus without first-line Daratumumab. Deep hematologic and organ responses (CR/VGPR) were higher in the Daratumumab group and strongly associated with improved survival. Conclusion Daratumumab as part of the first two lines of therapy is associated with superior survival and deeper hematological and organ response in patients with advanced cardiac AL amyloidosis, supporting its broader application.

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Publication Details

Journal
Cardio-Oncology
Published
2026-09-25
DOI
https://doi.org/10.1186/s40959-026-00580-1
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
Type
article
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article

Benefit of daratumumab-based treatment in advanced cardiac stage light chain amyloidosis

Max J. Rieger, Lara Gullo, Dominik C. Benz, Bernhard Gerber et al.
Cardio-Oncology
Amyloidosis: Diagnosis, Treatment, Outcomes
article

Benefit of daratumumab-based treatment in advanced cardiac stage light chain amyloidosis

Max J. Rieger, Lara Gullo, Dominik C. Benz, Bernhard Gerber, Martina Bertschinger, Holger W. Auner, Caroline Cicin‐Sain, Reto Kühne, Rahel Schwotzer, Natallia Laptseva, Sabine Gerull, Andreas J. Flammer, R Battegay, Thomas Fehr, Marie-Noëlle Kronig, Julia Bee, Carmen de Ramon Ortiz, Ulrich Mey, Harald Seeger, Christian Kuhn, Felicitas Hitz, Michele Hoffmann, Veronika Ballova, Thomas Pabst, Adrian Schmidt
article en

Abstract

Abstract Background Advanced cardiac-stage light chain (AL) amyloidosis has a poor prognosis. Other than in early-stage disease, Daratumumab’s role is less well established. Methods In this multi-center, retrospective study we analyzed patients with newly diagnosed AL amyloidosis with cardiac involvement and baseline NT-proBNP of ≥ 1800 ng/l, enrolled in the Swiss Amyloidosis Registry. We compared overall survival (OS), progression-free survival 1 and 2 (PFS), and treatment response in patients receiving Daratumumab as first- or second-line therapy to those who never received Daratumumab. Results Seventy six patients were included, median baseline NT-proBNP was 4’180 ng/l. 25 and 51 patients had 2012 revised Mayo stage III and IV disease. Patients treated with Daratumumab showed significantly higher OS and improved PFS2 compared with those who never received Daratumumab (2y-OS 75.8% vs. 52.2%, log-rank < 0.05, 2y-PFS2 75.4% vs. 58.4%, log-rank: 0.068). No significant difference was observed between patients treated with versus without first-line Daratumumab. Deep hematologic and organ responses (CR/VGPR) were higher in the Daratumumab group and strongly associated with improved survival. Conclusion Daratumumab as part of the first two lines of therapy is associated with superior survival and deeper hematological and organ response in patients with advanced cardiac AL amyloidosis, supporting its broader application.

Cardio-Oncology
University of Zurich (CH), University of St.Gallen (CH), Kantonsspital St. Gallen (CH), Triemli Hospital (CH), University Hospital of Bern (CH), University Hospital of Basel (CH), Ente Ospedaliero Cantonale (CH), University Hospital of Zurich (CH), University Hospital of Geneva (CH), Kantonsspital Baden (CH), Kantonsspital Graubünden (CH), Spital Thurgau (Switzerland) (CH), Kantonsspital Aarau (CH), Università della Svizzera italiana (CH), University of Lausanne (CH)
No poverty
Openalex Percentile: Top 19%
Amyloidosis: Diagnosis, Treatment, Outcomes
DOIOpen Access PDFOpenAlex
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