A Dose-Tailored Anti-Plasma Cell Regimen Lowers the Mortality of Late-Stage Cardiac Amyloidosis

Abstract

A major obstacle to using current guideline-recommended chemotherapy in patients with advanced light-chain cardiac amyloidosis (LCCA) is their intolerance to standard drug dosages. The study aimed to assess the efficacy and safety of the dose-tailored BD and DBD regimen proposed by our team for patients with LCCA at Mayo Stage III. A total of 119 patients who met the inclusion and exclusion criteria for cardiac amyloidosis were recruited and divided into three groups: group A, group B, and group C who received supportive therapy, dose-tailored BD regimen, and dose-tailored DBD regimen, respectively. Survival rate and time, hematologic and cardiac response, and adverse events were evaluated during a median follow-up of 30.2 months. No significant differences in baseline characteristics were found among the three groups. Group B and C showed increased survival rates and time compared to group A. Group C showed improved hematologic and cardiac responses relative to group B. Additionally, group C showed fewer adverse events related to chemotherapy compared to group B. Both dose-tailored BD and DBD regimens increased survival rates and time in advanced LCCA patients, with the dose-tailored DBD regimen demonstrating superior efficacy and safety. Further randomized clinical trials are needed to confirm these preliminary findings.

Keywords: cardiac amyloidosis; cardiac response; dose‐tailored anti‐plasma cell regimen; heart failure; hematologic response.