Single-center clinical efficacy analysis of 16 cases of primary light chain cardiac amyloidosis including stage IIIb patients.

IF 3.2 4区 医学 Q2 MEDICINE, RESEARCH & EXPERIMENTAL Clinical and Experimental Medicine Pub Date : 2025-03-16 DOI:10.1007/s10238-025-01616-z
Jing Wang, Shaojie Ye, Huimei Guo, Songying Zhao, Jia Liu, JiangBo Zhang, Jianmei Xu, Xi Su, Luoming Hua, Hua Xue
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Abstract

Objective: To investigate the clinical efficacy of daratumumab versus bortezomib in patients with systemic light chain amyloidosis (pAL) with cardiac involvement, particularly those at stage IIIb.

Methods: Retrospective analysis of 16 AL patients with cardiac involvement, two groups of patients received treatment primarily with bortezomib and daratumumab, respectively. The hematologic remission rate, cardiac response rate, survival and adverse reactions of the two groups were analyzed.

Results: Among the 16 patients, 4 were classified as Mayo 2004 stage IIIa and 6 as stage IIIb. The hematological response rate and cardiac organ response rate were higher in the daratumumab group compared to the bortezomib group (71% vs. 33%; 57% vs. 11%). With a median follow-up of 12 months, the median progression-free survival (PFS) and overall survival (OS) were superior in the daratumumab group (not reached vs. 6 months, P = 0.022; 27.8 months vs. 21.7 months, P = 0.232).Specifically, among the 6 stage IIIb patients, the daratumumab group demonstrated higher hematological and cardiac response rates (66% vs. 0%; 66% vs. 0%).

Conclusions: For patients with AL amyloidosis and cardiac involvement,including those at stage IIIb, daratumumab-based regimens offer benefits in terms of hematological remission, cardiac response, and progression-free survival, with comparable tolerability to bortezomib.

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来源期刊
Clinical and Experimental Medicine
Clinical and Experimental Medicine 医学-医学:研究与实验
CiteScore
4.80
自引率
2.20%
发文量
159
审稿时长
2.5 months
期刊介绍: Clinical and Experimental Medicine (CEM) is a multidisciplinary journal that aims to be a forum of scientific excellence and information exchange in relation to the basic and clinical features of the following fields: hematology, onco-hematology, oncology, virology, immunology, and rheumatology. The journal publishes reviews and editorials, experimental and preclinical studies, translational research, prospectively designed clinical trials, and epidemiological studies. Papers containing new clinical or experimental data that are likely to contribute to changes in clinical practice or the way in which a disease is thought about will be given priority due to their immediate importance. Case reports will be accepted on an exceptional basis only, and their submission is discouraged. The major criteria for publication are clarity, scientific soundness, and advances in knowledge. In compliance with the overwhelmingly prevailing request by the international scientific community, and with respect for eco-compatibility issues, CEM is now published exclusively online.
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