Cardiac Response Dynamics in Newly Diagnosed Light-Chain Amyloidosis Patients With Early and High-Quality Hematologic Response.

BACKGROUND

The goal of hematological response has been well established in the treatment of systemic light chain amyloidosis. However, the pattern of cardiac response remains unknown.

OBJECTIVES

This study was designed to investigate the cardiac response dynamics in patients with an early and high-quality hematological response.

METHODS

This retrospective study included newly diagnosed patients who achieved a hematological response of very good partial response or better within 3 months after treatment beginning. Four cardiac response criteria were tested at fixed time points (3, 6, 12, and 24 months after therapy initiation): cardiac complete response, cardiac very good partial response, cardiac partial response (carPR), and cardiac no response.

RESULTS

A total of 201 patients were included, with the median follow-up 37.0 months (Q1-Q3: 18.0-56.5 months). The cardiac response reached a plateau at 24 months, while cardiac complete response, cardiac very good partial response, and carPR were achieved in 21.4% (28 of 131), 38.9% (51 of 131), and 20.6% (27 of 131) of the patients, respectively. At every fixed time point within 12 months after treatment initiation, patients who achieved a carPR or better consistently had better survival than did those who did not. At 3 months, an NT-proBNP concentration ≥3,716 pg/mL was the only factor associated with a decreased likelihood of achieving a carPR within 12 months (OR: 0.269; 95% CI: 0.137-0.512; 0.001).

CONCLUSIONS

This study emphasizes the importance of monitoring cardiac response dynamics for disease surveillance. In patients who achieve early and high-quality hematological response, it is important to achieve carPR within 12 months.