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Diagnosis and management of systemic light chain AL amyloidosis.
Pharmacology & Therapeutics ( IF 12.0 ) Pub Date : 2020-06-17 , DOI: 10.1016/j.pharmthera.2020.107612
Divaya Bhutani 1 , Suzanne Lentzsch 1
Affiliation  

AL amyloidosis is a plasma cell disorder leading to the production and extracellular deposition of abnormal immunoglobulin light chains called amyloid. The pathogenesis of the disorder is driven by an abnormal plasma cell clone producing excessive monoclonal immunoglobulin light chains that undergo deposition in various organs of the body such as the heart, kidney, and gastrointestinal tract. The outcome of the disease remains poor with significant morbidity and mortality associated with organ dysfunction. In this review, we describe the current standard diagnostic features, prognosis, and current treatment paradigm of the disease.



中文翻译:

全身轻链AL淀粉样变性的诊断和处理。

AL淀粉样变性是浆细胞疾病,导致异常免疫球蛋白轻链(称为淀粉样蛋白)的产生和细胞外沉积。该疾病的发病机理是由异常的浆细胞克隆驱动的,该克隆产生过量的单克隆免疫球蛋白轻链,这些轻链在人体的各个器官(如心脏,肾脏和胃肠道)中沉积。该疾病的结果仍然很差,与器官功能障碍有关的发病率和死亡率均很高。在这篇综述中,我们描述了疾病的当前标准诊断特征,预后和当前治疗范例。

更新日期:2020-06-28
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