轻链（AL）淀粉样变是一种浆细胞肿瘤类型，其产生异常的单克隆免疫球蛋白轻链并将它们沉积在组织中导致器官损伤。除了现有治疗方案，包括自体干细胞移植，还需要其他方法来根除异常浆细胞和淀粉样组织沉积物。AL淀粉样变的治疗策略主要基于在多发性骨髓瘤中有效的药物。达拉莫单抗连同蛋白酶体抑制剂和皮质类固醇已成为AL淀粉样变的标准治疗。另一个有吸引力的方法是解体淀粉样沉积物，以期潜在地逆转疾病造成的损伤。对于CAEL-101和birtamimab，这取得了有前途的成果。虽然仍处于早期阶段，但在管道中的新型治疗选择，包括抗体药物联合物、双特异性T细胞诱导剂和嵌合抗原受体T细胞疗法，可能在未来多样化AL淀粉样变治疗武器库。版权所有©2023 Elsevier B.V.。保留所有权利。

Light-chain (AL) amyloidosis is a type of plasma cell neoplasm with abnormal monoclonal immunoglobulin light-chain production and their subsequent deposition in tissues causing end-organ damage. In addition to existing treatments including autologous stem cell transplantation, there is a need for other approaches for eradicating abnormal plasma cells and amyloid tissue deposits. Treatment strategies of AL amyloidosis are mostly based on medications that are effective in multiple myeloma due to similar cell of origin. Daratumumab along with proteasome inhibitors and corticosteroids has become standard of care for AL amyloidosis. Another appealing approach is disassembling amyloid deposits with hope to potentially reverse the damage done by the disease. This was met with promising results for CAEL-101 and birtamimab. Although still in early stages, novel treatment options in pipeline, including antibody-drug conjugates, bispecific T-cell engagers, and chimeric antigen receptor T cell therapy may diversify the treatment armamentarium of AL amyloidosis in the future.Copyright © 2023 Elsevier B.V. All rights reserved.