Am J Med. 2022 Jan 23:S0002-9343(22)00027-4. doi: 10.1016/j.amjmed.2022.01.007. Online ahead of print.
AL amyloidosis is challenging to diagnose and it should be considered a cardiac emergency. There has been a great deal of advances in the treatment of AL amyloidosis from initial descriptions of melphalan therapy until the recent approval of the first AL amyloidosis specific drug (daratumumab). Comprehension of the pathophysiology and biology of AL amyloidosis is key to understand the major therapeutic targets in which light chain stability remains as a major key target of therapy. Organ dysfunction is a result not only from disruption of organ architecture, but also direct cellular toxicity. Novel antiplasma cell agents for AL like isatuximab (anti CD-38 monoclonal antibody), belantamab (anti-BCMA monoclonal antibody) and elotuzumab (anti-SLAMF7 monoclonal antibody are currently under investigation. Both diagnostic and therapeutic advances make the future of AL management bright acknowledging the complexity of this patient population and focusing on a multidisciplinary approach.