Future Developments in Light Chain Amyloidosis Management - 07/05/22

Doi : 10.1016/j.amjmed.2022.01.007

Mario Rodriguez, MD ^a,^⁎ , Daniel Lenihan, MD ^a, Giampaolo Merlini, MD ^b
^a Cardio-Oncology Center of Excellence, Cardiovascular Division, Washington University School of Medicine, St. Louis, Mo
^b Amyloidosis Research and Treatment Center, Foundation IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy

^⁎Requests for reprints should be addressed to Mario Rodriguez, MD, Cardiovascular Division, 660 S Euclid Ave, Campus Box 8086, St Louis, MO, 63110.Cardiovascular Division660 S Euclid Ave, Campus Box 8086St LouisMO63110

Abstract

Light chain (AL) amyloidosis is challenging to diagnose, and it should be considered a cardiac emergency. There have 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 crucial to understanding 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 while acknowledging the complexity of this patient population and focusing on a multidisciplinary approach.

El texto completo de este artículo está disponible en PDF.

Keywords : Amyloidosis, Cardiomyopathy, Myeloma

Esquema

Introduction

Key Pathobiological Principles and Targets

Advances in Diagnosis

Advances in Therapy

Conclusion

Cme Statement

	Funding: None.
	Conflicts of Interest: MR reports none. DL reports research support from Alnylam, Eidos, Prothena, Pfizer, and Jazz Pharmaceuticals and consulting fees from Bristol-Myers Squibb, Ipsen Biopharmaceuticals, Prothena, Acorda, Lilly (Eli) USA, and Roche. GM reports consulting fees from Janssen, Takeda, and Abbvie.
	Authorship: All authors had access to the data and a role in writing this manuscript.