Advances in the Therapy of Light Chain Cardiac Amyloidosis.

IF 2.5 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS
Heart Failure Clinics Pub Date : 2024-07-01 Epub Date: 2025-01-30 DOI:10.1016/j.hfc.2024.09.005
Giuseppe Palmiero, Paolo Milani, Federica Verrillo, Emanuele Monda, Marco Merlo, Michele Emdin, Giuseppe Limongelli, Giovanni Palladini
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引用次数: 0

Abstract

Systemic light chain (AL) amyloidosis stems from abnormal production of amyloidogenic immunoglobulin light chains by plasma cells or B-cell disorders. It can present locally or systemically, with systemic forms posing significant mortality risks, especially if cardiac involvement is present. Delayed diagnosis due to nonspecific symptoms leads to progressive organ dysfunction. Early recognition is vital for timely treatment, aiming to suppress amyloid production and reduce organ damage, thus promoting recovery and improving survival rates. This review article provides a comprehensive overview of the recent advancements in medical therapy for patients with AL cardiac amyloidosis.

轻链型心脏淀粉样变性的治疗进展。
系统性轻链(AL)淀粉样变性源于浆细胞或b细胞异常产生淀粉样免疫球蛋白轻链。它可以表现为局部或全身性,全身性形式具有显著的死亡风险,特别是如果存在心脏受累。由于非特异性症状而延误诊断导致进行性器官功能障碍。早期识别对于及时治疗至关重要,旨在抑制淀粉样蛋白的产生,减少器官损伤,从而促进恢复和提高生存率。本文综述了AL型心脏淀粉样变性的医学治疗的最新进展。
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来源期刊
Heart Failure Clinics
Heart Failure Clinics CARDIAC & CARDIOVASCULAR SYSTEMS-
CiteScore
5.40
自引率
2.90%
发文量
72
期刊介绍: Heart failure is quickly becoming the most pressing health problem in the United States. Heart Failure Clinics provides practical, concise and easy-to-read coverage that encompasses everything you need to know about the current literature. Topics include acute decompensated heart failure, arrhythmias and sudden cardiac death, ventricular dysfunction, comorbidities of heart failure, diastolic dysfunction, genetic evaluation, ischemic heart disease, pulmonary arterial hypertension, and surgery/transplantation.
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