Purpose of Review

Light chain (AL) amyloidosis is an insidious progressive disease which results in significant morbidity and inevitable mortality if not diagnosed and treated promptly. This review will highlight recent developments and summarize critical clinical points and updated practice changes for the clinician in 2020.

Recent Findings

Comparative analyses of staging systems, updated prognostic tools, and treatment response criteria now allow for improved patient stratification and treatment decisions; the role of minimal residual disease in response assessment is still being assessed. Clinical and genetic predictors for long-term survivors have been highlighted. Standard-of-care front-line bortezomib and the integration of anti-CD38 monoclonal antibodies in the relapsed disease have transformed treatment approach in recent years. Various clinical trials in the pipeline include novel anti-plasma cell therapies and therapies directed against amyloid deposits which promise to further advance the treatment landscape.

Summary

Diagnosis, response assessment, and treatment paradigms for AL amyloidosis have evolved significantly in the past 15 years, translating into superior outcomes and increased chances of long-term survival for AL amyloidosis.

中文翻译：

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AL淀粉样变性的诊断和管理方面的最新进展。

审查目的

轻链（AL）淀粉样变性病是一种隐匿性进行性疾病，如果不及时诊断和治疗，会导致高发病率和不可避免的死亡率。这篇综述将重点介绍2020年的最新进展，并总结关键临床要点和临床医生的最新实践变更。

最近的发现

现在，通过对分期系统，更新的预后工具和治疗反应标准进行比较分析，可以改善患者的分层和治疗决策；最小残留疾病在反应评估中的作用仍在评估中。长期幸存者的临床和遗传预测因子已得到强调。近年来，护理标准一线硼替佐米和抗CD38单克隆抗体在复发性疾病中的整合已改变了治疗方法。正在进行的各种临床试验包括新颖的抗浆细胞疗法和针对淀粉样蛋白沉积物的疗法，有望进一步改善治疗前景。

概要

在过去的15年中，AL淀粉样变性的诊断，反应评估和治疗范例已发生了显着变化，转化为AL淀粉样变性的优异结果和长期生存的机会增加了。