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[轻链型淀粉样变性]

[Light chain amyloidosis].

作者信息

Hegenbart Ute, Aus dem Siepen Fabian, Schönland Stefan

机构信息

Amyloidose-Zentrum, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Deutschland.

Klinik für Kardiologie, Angiologie und Pneumologie, Universitätsklinikum Heidelberg, Heidelberg, Deutschland.

出版信息

Inn Med (Heidelb). 2023 Sep;64(9):842-847. doi: 10.1007/s00108-023-01568-0. Epub 2023 Aug 4.

Abstract

Light chain amyloidosis (AL) is a rare protein deposition disease. It is caused by a clonal plasma cell or B‑cell disease in the bone marrow. With the exception of the central nervous system, all organs can be affected by amyloid deposits. Cardiac involvement is the most frequent organ manifestation that leads to significantly increased mortality when it is diagnosed at an advanced stage. The causal treatment of AL amyloidosis is reduction of amyloidogenic light chains by chemotherapy. Early diagnosis of the disease is essential to reduce early mortality, to effectively treat patients and to prevent further deterioration of organ function. New treatment approaches for AL amyloidosis are aimed at inhibiting amyloid formation or degradation of amyloid in organs.

摘要

轻链淀粉样变性(AL)是一种罕见的蛋白质沉积疾病。它由骨髓中的克隆性浆细胞或B细胞疾病引起。除中枢神经系统外,所有器官均可受到淀粉样沉积物的影响。心脏受累是最常见的器官表现,在晚期诊断时会导致死亡率显著增加。AL淀粉样变性的病因治疗是通过化疗减少淀粉样轻链。疾病的早期诊断对于降低早期死亡率、有效治疗患者以及预防器官功能进一步恶化至关重要。AL淀粉样变性的新治疗方法旨在抑制淀粉样蛋白的形成或器官中淀粉样蛋白的降解。

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