Amyloid light chain (AL) amyloidosis is among the more common and more severe of the amyloidoses usually involving the slow proliferation of a bone-marrow-residing plasma cell (PC) clone and the secretion of unstable immunoglobulin-free light chains (FLC) that infiltrate peripheral tissues and result in detrimental end-organ damage. Disease presentation is rather vague, and the hallmark of treatment is early diagnosis before irreversible end-organ damage. Once diagnosed, treatment decision is transplant-driven whereby ~20% of patients are eligible for autologous stem cell transplantation (ASCT) with or without bortezomib-based induction. In the setting of ASCT-ineligibility, bortezomib plays a central role in upfront treatment with the recent addition of daratumumab to the current emerging standard of care. In general, management of AL amyloidosis is aimed at achieving deep, durable responses with very close monitoring for early detection of relapse/refractory disease. This article provides a comprehensive review of the management of patients with AL amyloidosis including goals of therapy, current treatment guidelines in the setting of both ASCT-eligibility and ineligibility, treatment response monitoring recommendations, toxicity management, and treatment of relapse/refractory disease.

淀粉样轻链 (AL) 淀粉样变性是一种较常见且较严重的淀粉样变性，通常涉及骨髓内浆细胞 (PC) 克隆的缓慢增殖和不稳定的无免疫球蛋白轻链 (FLC) 的分泌，渗透周围组织并导致有害的终末器官损伤。疾病表现相当模糊，治疗的特点是在不可逆的终末器官损伤之前进行早期诊断。一旦确诊，治疗决策将由移植决定，约 20% 的患者有资格接受自体干细胞移植 (ASCT)，无论是否采用基于硼替佐米的诱导。在不符合 ASCT 的情况下，硼替佐米在前期治疗中发挥着核心作用，最近将达雷妥尤单抗添加到当前新兴的护理标准中。一般来说，AL 淀粉样变性的治疗旨在通过密切监测以早期发现复发/难治性疾病来实现深度、持久的反应。本文全面回顾了 AL 淀粉样变性患者的治疗，包括治疗目标、ASCT 资格和不合格情况下的当前治疗指南、治疗反应监测建议、毒性管理和复发/难治性疾病的治疗。