TY - JOUR
T1 - Treatment of AL Amyloidosis
T2 - Mayo Stratification of Myeloma and Risk-Adapted Therapy (mSMART) Consensus Statement 2020 Update
AU - Muchtar, Eli
AU - Dispenzieri, Angela
AU - Gertz, Morie A.
AU - Kumar, Shaji K.
AU - Buadi, Francis K.
AU - Leung, Nelson
AU - Lacy, Martha Q.
AU - Dingli, David
AU - Ailawadhi, Sikander
AU - Bergsagel, P. Leif
AU - Fonseca, Rafael
AU - Hayman, Suzanne R.
AU - Kapoor, Prashant
AU - Grogan, Martha
AU - Abou Ezzeddine, Omar F.
AU - Rosenthal, Julie L.
AU - Mauermann, Michelle
AU - Siddiqui, Mustaqueem
AU - Gonsalves, Wilson I.
AU - Kourelis, Taxiarchis V.
AU - Larsen, Jeremy T.
AU - Reeder, Craig B.
AU - Warsame, Rahma
AU - Go, Ronald S.
AU - Murray, David L.
AU - McPhail, Ellen D.
AU - Dasari, Surendra
AU - Jevremovic, Dragan
AU - Kyle, Robert A.
AU - Lin, Yi
AU - Lust, John A.
AU - Russell, Stephen J.
AU - Hwa, Yi Lisa
AU - Fonder, Amie L.
AU - Hobbs, Miriam A.
AU - Rajkumar, S. Vincent
AU - Roy, Vivek
AU - Sher, Taimur
N1 - Publisher Copyright:
© 2021 Mayo Foundation for Medical Education and Research
PY - 2021/6
Y1 - 2021/6
N2 - Immunoglobulin 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.
AB - Immunoglobulin 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.
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U2 - 10.1016/j.mayocp.2021.03.012
DO - 10.1016/j.mayocp.2021.03.012
M3 - Review article
C2 - 34088417
AN - SCOPUS:85107161252
SN - 0025-6196
VL - 96
SP - 1546
EP - 1577
JO - Mayo Clinic proceedings
JF - Mayo Clinic proceedings
IS - 6
ER -