[Recent advances in management of systemic AL amyloidosis]

Systemic AL amyloidosis is a progressive disorder characterized by deposition of immunoglobulin light chain-derived amyloid fibrils in multiple organs, resulting in diverse clinical manifestations. A definitive diagnosis requires histopathological confirmation of amyloid deposition, and delayed diagnosis is closely associated with poor outcomes. The current standard frontline therapy for newly diagnosed patients is the combination of daratumumab, cyclophosphamide, bortezomib, and dexamethasone, which has demonstrated high hematologic and organ response rates. Autologous hematopoietic stem cell transplantation can induce deep and durable hematologic responses; however, transplant-related mortality remains higher than in multiple myeloma, necessitating careful patient selection. For relapsed or refractory cases, conventional anti-myeloma agents such as lenalidomide, pomalidomide, and ixazomib remain options, while emerging therapies including venetoclax and BCMA-targeted approaches show promising efficacy. Investigational strategies targeting amyloid fibrils are also under active development, although their clinical benefit remains to be fully established. Advances in early diagnosis and novel therapeutic approaches are essential to improve outcomes in systemic AL amyloidosis.