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Predicting Early Mortality in Newly Diagnosed AL Amyloidosis: Development and Validation of the PACE Score for Daratumumab-Treated Patients - PubMed

4 days ago
  • #AL amyloidosis
  • #early mortality
  • #daratumumab
  • The study developed and validated the PACE score to predict early mortality (within 6 months) in newly diagnosed AL amyloidosis patients treated with daratumumab-based regimens.
  • Early mortality occurred in 10.6% of the 339 patients studied across eight centers.
  • Four independent predictors of early mortality were identified: NT-proBNP > 5300 pg/mL, Age > 75 years, absence of t(11;14) on cytogenetics, and ECOG performance status.
  • The PACE score stratified patients into low (1.2% mortality), intermediate (11.7%), and high-risk (50.0%) groups, showing superior discrimination compared to existing staging systems.
  • External validation confirmed the PACE score's robustness, with mortality rates of 4.2%, 15.25%, and 37.04% across risk groups.
  • The PACE score aids in patient counseling, clinical trial design, and identifying high-risk patients needing intensive monitoring and novel therapies.