Light-chain (AL) amyloidosis is a rare plasma cell disorder characterized by the deposition of misfolded immunoglobulin light chains in target organs, leading to multi-organ dysfunction. Treatment approaches have historically mirrored but lagged behind those of multiple myeloma (MM). Recent advancements in MM immunotherapy are gradually being evaluated and adopted in AL amyloidosis. This review explores the current state of immunotherapeutic strategies in AL amyloidosis, including monoclonal antibodies, antibody–drug conjugates, bispecific antibodies, and chimeric antigen receptor T-cell therapy. We discuss the unique challenges and prospects of these therapies in AL amyloidosis, including the exposure of frail AL amyloidosis patients to immune-mediated toxicities such as cytokine release syndrome (CRS) and immune effector-cell-associated neurotoxicity syndrome (ICANS), as well as their efficacy in promoting rapid and deep hematologic responses. Furthermore, we highlight the need for international initiatives and compassionate programs to provide access to these promising therapies and address critical unmet needs in AL amyloidosis management. Finally, we discuss future directions, including optimizing treatment sequencing and mitigating toxicities, to improve outcomes for AL amyloidosis patients.
轻链（AL）型淀粉样变性是一种罕见的浆细胞疾病，其特征是错误折叠的免疫球蛋白轻链在靶器官沉积，导致多器官功能障碍。其治疗方法历来参照多发性骨髓瘤（MM），但发展相对滞后。近年来，多发性骨髓瘤免疫治疗领域的进展正逐步在AL型淀粉样变性中得到评估和应用。本综述探讨了AL型淀粉样变性免疫治疗策略的现状，包括单克隆抗体、抗体-药物偶联物、双特异性抗体以及嵌合抗原受体T细胞疗法。我们讨论了这些疗法在AL型淀粉样变性中面临的独特挑战与前景，包括体质虚弱的AL型淀粉样变性患者面临细胞因子释放综合征（CRS）和免疫效应细胞相关神经毒性综合征（ICANS）等免疫介导毒性的风险，以及这些疗法在促进快速深度血液学缓解方面的疗效。此外，我们强调需要通过国际倡议和同情用药计划，使患者能够获得这些前景广阔的治疗方法，并解决AL型淀粉样变性管理中的关键未满足需求。最后，我们探讨了未来的发展方向，包括优化治疗顺序和减轻毒性，以改善AL型淀粉样变性患者的预后。
肿瘤(癌症)患者之家