文章：

免疫球蛋白轻链淀粉样变性诊断与治疗流程图（2021版）

Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2021

原文发布日期：2021-05-15

DOI: 10.1038/s41408-021-00483-7

类型: Review Article

开放获取: 是

英文摘要：

Immunoglobulin light chain amyloidosis (AL) commonly presents with nephrotic range proteinuria, heart failure with preserved ejection fraction, nondiabetic peripheral neuropathy, unexplained hepatomegaly or diarrhea, and should be considered in patients presenting with these symptoms. More importantly, patients being monitored for smoldering multiple myeloma and a monoclonal gammopathy of undetermined significance (MGUS) are at risk for developing AL amyloidosis. MGUS and myeloma patients that have atypical features, including unexplained weight loss; lower extremity edema, early satiety, and dyspnea on exertion should be considered at risk for light chain amyloidosis. Overlooking the diagnosis of light chain amyloidosis leading to therapy delay is common, and it represents an error of diagnostic consideration. Herein we provide a review of established and investigational treatments for patients with AL amyloidosis and provide algorithms for workup and management of these patients.
 

摘要翻译： 

免疫球蛋白轻链淀粉样变性（AL）通常表现为肾病范围蛋白尿、射血分数保留的心力衰竭、非糖尿病性周围神经病变、不明原因的肝肿大或腹泻，出现这些症状的患者应考虑此病。更重要的是，正在监测中的冒烟型多发性骨髓瘤和意义未明的单克隆免疫球蛋白病（MGUS）患者有发展为AL淀粉样变性的风险。具有非典型特征的MGUS和骨髓瘤患者，包括不明原因的体重减轻、下肢水肿、早饱感和劳力性呼吸困难，应被视为轻链淀粉样变性的高危人群。忽视轻链淀粉样变性的诊断导致治疗延误的情况很常见，这属于诊断考量上的失误。本文旨在综述AL淀粉样变性的现有及研究中的治疗方法，并为这类患者的检查与管理提供诊疗流程。

原文链接：

Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2021