TY - JOUR
T1 - How i manage systemic light chain amyloidosis
AU - Kaufman, Gregory P.
AU - Cerchione, Claudio
N1 - Publisher Copyright:
© 2021, Page Press Publications. All rights reserved.
PY - 2021/9
Y1 - 2021/9
N2 - Light chain amyloidosis (AL) is a protein deposition disorder with a heterogenous pattern of organ involvement and dysfunction that varies by affected patient. Often diagnosed late after the onset of symptoms, appropriate and correct diagnosis, and prompt initial management, typically with anti-plasma cell therapy targeting the underlying cell producing the aberrant light chain protein, can lead to significant improvement in patient symptoms, organ function and lifespan. With recent publication of phase III studies focused on AL, and a changing regulatory landscape providing greater availability of novel therapies, the management of AL is in some ways more complex with greater options to consider. In this clinical review, a discussion of the diagnosis, staging, and goals of therapy transitions to a focus on contemporary management questions to outline our clinical approach to multi-disciplinary management and long term follow up for patients with suspected or confirmed AL.
AB - Light chain amyloidosis (AL) is a protein deposition disorder with a heterogenous pattern of organ involvement and dysfunction that varies by affected patient. Often diagnosed late after the onset of symptoms, appropriate and correct diagnosis, and prompt initial management, typically with anti-plasma cell therapy targeting the underlying cell producing the aberrant light chain protein, can lead to significant improvement in patient symptoms, organ function and lifespan. With recent publication of phase III studies focused on AL, and a changing regulatory landscape providing greater availability of novel therapies, the management of AL is in some ways more complex with greater options to consider. In this clinical review, a discussion of the diagnosis, staging, and goals of therapy transitions to a focus on contemporary management questions to outline our clinical approach to multi-disciplinary management and long term follow up for patients with suspected or confirmed AL.
KW - Amyloidosis
KW - Light chain amyloidosis
KW - Management
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U2 - 10.4081/hr.2021.9383
DO - 10.4081/hr.2021.9383
M3 - Article
AN - SCOPUS:85118133577
SN - 2038-8322
VL - 13
JO - Hematology Reports
JF - Hematology Reports
IS - 3
ER -