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.

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予以考虑。更重要的是，正在监测冒烟型多发性骨髓瘤和意义不明的单克隆丙种球蛋白病 (MGUS) 的患者有发生 AL 淀粉样变性的风险。具有非典型特征的 MGUS 和骨髓瘤患者，包括无法解释的体重减轻；应考虑下肢水肿、早饱和劳力性呼吸困难有轻链淀粉样变性的风险。忽视轻链淀粉样变性的诊断导致治疗延迟是常见的，它代表了诊断考虑的错误。