Cardiac Response Dynamics in Newly Diagnosed Light-Chain Amyloidosis Patients With Early and High-Quality Hematologic Response
Cheng-yang Xu, MD,
Ai Guan, MD,
Lu Zhang, MD,
Zhuang Tian, MD,
Dao-bin Zhou, MD,
Kai-ni Shen, MD,
Jian Li, MD
Affiliations
Cheng-yang Xu, MD
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Ai Guan, MD
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Lu Zhang, MD
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Zhuang Tian, MD
Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Dao-bin Zhou, MD
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Kai-ni Shen, MD
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; Address for correspondence: Dr Kai-ni Shen, Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing 100730, People's Republic of China.
Jian Li, MD
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; State Key Laboratory of Common Mechanism Research for Major Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; Dr Jian Li, Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing 100730, People's Republic of China.
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; P < 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.
