Nirija Ranjit Anderson, Dariusz Korczyk, Peter Mollee
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引用次数: 0
Abstract
Introduction: Light chain (AL) amyloidosis is a rare and complex disease which can affect various systems of the body. In common with many rare and multisystemic diseases, the breadth of diagnostic, clinical, and supportive expertise required to care for such patients is best met by a multidisciplinary team.
Areas covered: We outline different phases of the patients' journey, including diagnosis, staging, treatment, and response assessment, to highlight common clinical issues best resolved by a multidisciplinary approach.
Expert opinion: To extend the benefit of multidisciplinary care to the majority of patients with AL amyloidosis, innovative healthcare models such as telehealth and multisite multidisciplinary team meetings need to be implemented. The need for a multidisciplinary approach where such a wide array of healthcare skills is required also highlights the shortcomings of our current diagnostic and monitoring assays. Better access to diagnostic and subtyping assays is necessary. The ability to characterize and measure the causative amyloidogenic light chain as well as imaging techniques to accurately diagnose and monitor response to therapy is also needed and is currently an area of research focus.
简介轻链(AL)淀粉样变性是一种罕见而复杂的疾病,可影响人体的各个系统。与许多罕见的多系统疾病一样,护理这类患者所需的诊断、临床和支持性专业知识的广度最好由一个多学科团队来完成:我们概述了患者治疗过程中的不同阶段,包括诊断、分期、治疗和反应评估,以突出多学科方法最能解决的常见临床问题:专家观点:为了让大多数 AL 淀粉样变性患者享受到多学科治疗的益处,需要实施创新的医疗模式,如远程医疗和多地点多学科团队会议。多学科方法需要如此广泛的医疗保健技能,这也凸显了我们目前诊断和监测化验的不足之处。有必要更好地提供诊断和亚型分析方法。此外,还需要对致病淀粉样蛋白轻链进行特征描述和测量的能力,以及准确诊断和监测治疗反应的成像技术,这也是目前研究的重点领域。
期刊介绍:
Advanced molecular research techniques have transformed hematology in recent years. With improved understanding of hematologic diseases, we now have the opportunity to research and evaluate new biological therapies, new drugs and drug combinations, new treatment schedules and novel approaches including stem cell transplantation. We can also expect proteomics, molecular genetics and biomarker research to facilitate new diagnostic approaches and the identification of appropriate therapies. Further advances in our knowledge regarding the formation and function of blood cells and blood-forming tissues should ensue, and it will be a major challenge for hematologists to adopt these new paradigms and develop integrated strategies to define the best possible patient care. Expert Review of Hematology (1747-4086) puts these advances in context and explores how they will translate directly into clinical practice.