文章：

免疫球蛋白轻链淀粉样变性诊断和治疗流程图2018

Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2018

原文发布日期：2018-05-23

DOI: 10.1038/s41408-018-0080-9

类型: Current Treatment Algorithm

开放获取: 是

英文摘要：

Immunoglobulin light chain amyloidosis (AL) should be considered in any patient that presents to a cancer care provider with nephrotic range proteinuria, heart failure with preserved ejection fraction, non-diabetic peripheral neuropathy, unexplained hepatomegaly or diarrhea. 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. Algorithms will be provided on how to evaluate patients with suspected AL amyloid as well as how to manage patients referred from other medical specialties with biopsy-proven amyloid. An organized stepwise approach to the treatment of patients with light chain amyloidosis, including established and investigational therapies, will be reviewed.

摘要翻译： 

任何向癌症治疗机构就诊、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、不明原因肝肿大或腹泻的患者，都应考虑免疫球蛋白轻链淀粉样变性（AL）的可能性。更重要的是，正在监测的冒烟型多发性骨髓瘤和意义未明的单克隆丙种球蛋白病（MGUS）患者有发生AL淀粉样变性的风险。MGUS和骨髓瘤患者若出现非典型特征，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迟的情况很常见，这属于诊断考量失误。本文将提供评估疑似AL淀粉样变性患者的流程指南，以及如何处理从其他医学专科转诊的经活检证实为淀粉样变性的患者。同时将回顾轻链淀粉样变性患者的系统化阶梯治疗方案，包括已确立的治疗方法和研究性疗法。

原文链接：

Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2018