• Eli Muchtar, Angela Dispenzieri, Morie A Gertz, Shaji K Kumar, Francis K Buadi, Nelson Leung, Martha Q Lacy, David Dingli, Sikander Ailawadhi, P Leif Bergsagel, Rafael Fonseca, Suzanne R Hayman, Prashant Kapoor, Martha Grogan, Omar F Abou Ezzeddine, Julie L Rosenthal, Michelle Mauermann, Mustaqueem Siddiqui, Wilson I Gonsalves, Taxiarchis V Kourelis, Jeremy T Larsen, Craig B Reeder, Rahma Warsame, Ronald S Go, David L Murray, Ellen D McPhail, Surendra Dasari, Dragan Jevremovic, Robert A Kyle, Yi Lin, John A Lust, Stephen J Russell, Yi Lisa Hwa, Amie L Fonder, Miriam A Hobbs, S Vincent Rajkumar, Vivek Roy, and Taimur Sher.
• Division of Hematology, Mayo Clinic, Rochester, MN. Electronic address: muchtar.eli@mayo.edu.
• Mayo Clin. Proc. 2021 Jun 1; 96 (6): 1546-1577.

AbstractImmunoglobulin light chain (AL) amyloidosis is a clonal plasma cell disorder leading to progressive and life-threatening organ failure. The heart and the kidneys are the most commonly involved organs, but almost any organ can be involved. Because of the nonspecific presentation, diagnosis delay is common, and many patients are diagnosed with advanced organ failure. In the era of effective therapies and improved outcomes for patients with AL amyloidosis, the importance of early recognition is further enhanced as the ability to reverse organ dysfunction is limited in those with a profound organ failure. As AL amyloidosis is an uncommon disorder and given patients' frailty and high early death rate, management of this complex condition is challenging. The treatment of AL amyloidosis is based on various anti-plasma cell therapies. These therapies are borrowed and customized from the treatment of multiple myeloma, a more common disorder. However, a growing number of phase 2/3 studies dedicated to the AL amyloidosis population are being performed, making treatment decisions more evidence-based. Supportive care is an integral part of management of AL amyloidosis because of the inherent organ dysfunction, limiting the delivery of effective therapy. This extensive review brings an updated summary on the management of AL amyloidosis, sectioned into the 3 pillars for survival improvement: early disease recognition, anti-plasma cell therapy, and supportive care.Copyright © 2021 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

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