Graded Cardiac Response Criteria for Patients With Systemic Light Chain Amyloidosis

Eli Muchtar, Angela Dispenzieri, Brendan Wisniowski, Giovanni Palladini, Paolo Milani, Giampaolo Merlini, Stefan Schönland, Kaya Veelken, Ute Hegenbart, Susan M. Geyer, Shaji K. Kumar, Efstathios Kastritis, Meletios A. Dimopoulos, Michaela Liedtke, Ronald Witteles, Vaishali Sanchorawala, Raphael Szalat, Heather Landau, Erica Petrlik, Suzanne LentzschAlexander Coltoff, Joan Bladé, Maria Teresa Cibeira, Oliver Cohen, Darren Foard, Ashutosh Wechalekar, Morie A. Gertz

Research output: Contribution to journalArticlepeer-review


PURPOSEBinary cardiac response assessment using cardiac biomarkers is prognostic in light chain amyloidosis. Previous studies suggested four-level cardiac responses using N-terminal prohormone of brain natiuretic peptide improves prognostic prediction. This study was designed to validate graded cardiac response criteria using N-terminal prohormone of brain natiuretic peptide/brain natiuretic peptide.PATIENTS AND METHODSThis retrospective, multicenter study included patients with light chain amyloidosis who achieved at least a hematologic partial response (PR) and were evaluable for cardiac response. Four response criteria were tested on the basis of natriuretic peptide response depth: cardiac complete response (CarCR), cardiac very good partial response (CarVGPR), cardiac PR (CarPR), and cardiac no response (CarNR). Response was classified as best response and at fixed time points (6, 12, and 24 months from therapy initiation). The study primary outcome was overall survival.RESULTS651 patients were included. Best CarCR, CarVGPR, CarPR, and CarNR were achieved in 16%, 26.4%, 22.9%, and 34.7% of patients, respectively. Patients in cardiac stage II were more likely to achieve CarCR than patients in cardiac stage IIIA and IIIB (22% v 13.5% v 3.2%; P <.001). A deeper cardiac response was associated with a longer survival (5-year overall survival 93%, 79%, 65%, and 33% for CarCR, CarVGPR, CarPR, and CarNR, respectively; P <.001). Fixed time-point analyses and time-varying covariates Cox regression analysis, to minimize survivorship bias, affirmed the independent survival advantage of deeper cardiac responses. Four-level response performed better than two-level response as early as 12 months from therapy initiation.CONCLUSIONGraded cardiac response criteria allow better assessment of cardiac improvement compared with the traditional binary response system. The study re-emphasizes the importance of early diagnosis, which increases the likelihood of deep cardiac responses.

Original languageEnglish (US)
Pages (from-to)1393-1403
Number of pages11
JournalJournal of Clinical Oncology
Issue number7
StatePublished - Mar 1 2023

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


Dive into the research topics of 'Graded Cardiac Response Criteria for Patients With Systemic Light Chain Amyloidosis'. Together they form a unique fingerprint.

Cite this