TY - JOUR
T1 - Outcomes of light-chain amyloidosis patients treated with first-line bortezomib
T2 - A collaborative retrospective multicenter assessment
AU - Israeli MM study group
AU - Gatt, Moshe E.
AU - Hardan, Izhar
AU - Chubar, Evgeni
AU - Suriu, Celia
AU - Tadmor, Tamar
AU - Shevetz, Olga
AU - Patachenco, Paulina
AU - Dally, Najib
AU - Yeganeh, Shay
AU - Ballan-Haj, Mouna
AU - Cohen, Yael
AU - Trestman, Svetlana
AU - Muchtar, Eli
AU - Magen, Hila
AU - Jakubinsky, Julia
AU - Avivi, Irit
N1 - Publisher Copyright:
© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
PY - 2016/2/1
Y1 - 2016/2/1
N2 - Light-chain amyloidosis (AL) is associated with low survival rates, particularly in patients with cardiac involvement. We evaluated the outcome of 73 consecutive, non-selected 'real-world' AL patients, treated with first-line bortezomib-based induction, focusing on the benefit of concurrent administration of alkylating agents. Most patients had renal (77%), cardiac (66%), or multiorgan (74%) involvement. Sixty-eight per cent (n = 50) received alkylating agent (mostly cyclophosphamide). Severe adverse events were seen in 45%, most evident in patients with cardiac involvement, with no increased toxicity in patients receiving an alkylator agent. Hematological response (HemR) was obtained in 77% of patients, including 33% very good partial responses and 19% complete responses. Age <70 yr, lack of cardiac and peripheral neurologic involvement, and co-administration of an alkylating agent were associated with significantly improved HemR. NYHA cardiac failure staging was the only independent factor affecting overall survival. Administration of an alkylating agent and the achievement of both HemR and organ response were associated with a statistically significant improved survival in those surviving the first 6 months of induction. First-line bortezomib-based regimen resulted in favorable response and survival in newly diagnosed patients. Co-administration of an alkylating agent improved outcome without increasing treatment-related toxicity.
AB - Light-chain amyloidosis (AL) is associated with low survival rates, particularly in patients with cardiac involvement. We evaluated the outcome of 73 consecutive, non-selected 'real-world' AL patients, treated with first-line bortezomib-based induction, focusing on the benefit of concurrent administration of alkylating agents. Most patients had renal (77%), cardiac (66%), or multiorgan (74%) involvement. Sixty-eight per cent (n = 50) received alkylating agent (mostly cyclophosphamide). Severe adverse events were seen in 45%, most evident in patients with cardiac involvement, with no increased toxicity in patients receiving an alkylator agent. Hematological response (HemR) was obtained in 77% of patients, including 33% very good partial responses and 19% complete responses. Age <70 yr, lack of cardiac and peripheral neurologic involvement, and co-administration of an alkylating agent were associated with significantly improved HemR. NYHA cardiac failure staging was the only independent factor affecting overall survival. Administration of an alkylating agent and the achievement of both HemR and organ response were associated with a statistically significant improved survival in those surviving the first 6 months of induction. First-line bortezomib-based regimen resulted in favorable response and survival in newly diagnosed patients. Co-administration of an alkylating agent improved outcome without increasing treatment-related toxicity.
KW - AL amyloidosis
KW - Bortezomib
KW - Chemotherapy
KW - Novel agents
UR - http://www.scopus.com/inward/record.url?scp=84955191567&partnerID=8YFLogxK
U2 - 10.1111/ejh.12558
DO - 10.1111/ejh.12558
M3 - ???researchoutput.researchoutputtypes.contributiontojournal.article???
C2 - 25827161
AN - SCOPUS:84955191567
SN - 0902-4441
VL - 96
SP - 136
EP - 143
JO - European Journal of Haematology
JF - European Journal of Haematology
IS - 2
ER -