title: Outcomes of light-chain amyloidosis patients treated with first-line bortezomib: a collaborative retrospective multicenter assessment.
abstract: 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.
authors.affiliation: Department of Hematology, Hadassah Medical Center, Jerusalem, Israel.
authors.forename: Moshe E
authors.initials: ME
authors.lastname: Gatt
authors.affiliation: Hematology Unit, Meir Medical Center, Kfar Saba, Israel.
authors.forename: Izhar
authors.initials: I
authors.lastname: Hardan
authors.affiliation: Department of Hematology, HaEmek Medical Center, Afula, Israel.
authors.forename: Evgeni
authors.initials: E
authors.lastname: Chubar
authors.affiliation: Department of Hematology, Western Galilee Hospital, Nahariya, Israel.
authors.forename: Celia
authors.initials: C
authors.lastname: Suriu
authors.affiliation: Hematology Unit, Bnai-Zion Medical Center, Haifa, Israel.
authors.forename: Tamar
authors.initials: T
authors.lastname: Tadmor
authors.affiliation: Department of Hematology, Kaplan Medical Center, Rehovot, Israel.
authors.forename: Olga
authors.initials: O
authors.lastname: Shevetz
authors.affiliation: Department of Hematology, Wolfson Medical Center, Holon, Israel.
authors.forename: Paulina
authors.initials: P
authors.lastname: Patachenco
authors.affiliation: Department of Hematology, Ziv Medical Center, Safed, Israel.
authors.forename: Najib
authors.initials: N
authors.lastname: Dally
authors.affiliation: Hematology Unit, Poria Medical Center, Tiberias, Israel.
authors.forename: Shay
authors.initials: S
authors.lastname: Yeganeh
authors.affiliation: Hematology Unit, Carmel Medical Center, Haifa, Israel.
authors.forename: Mouna
authors.initials: M
authors.lastname: Ballan-Haj
authors.affiliation: Department of Hematology, Sorasky Medical Center, Tel Aviv, Israel.
authors.forename: Yael
authors.initials: Y
authors.lastname: Cohen
authors.affiliation: Department of Hematology, Sorasky Medical Center, Tel Aviv, Israel.
authors.forename: Svetlana
authors.initials: S
authors.lastname: Trestman
authors.affiliation: Department of Hematology, Rabin Medical Center, Petah Tikva, Israel.
authors.forename: Eli
authors.initials: E
authors.lastname: Muchtar
authors.affiliation: Department of Hematology, Rabin Medical Center, Petah Tikva, Israel.
authors.forename: Hila
authors.initials: H
authors.lastname: Magen
authors.affiliation: Department of Hematology, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Julia
authors.initials: J
authors.lastname: Jakubinsky
authors.affiliation: Department of Hematology, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Irit
authors.initials: I
authors.lastname: Avivi
mesh_terms: D000368:Aged; D058227:Amyloidogenic Proteins / Q000097:blood*; D000686:Amyloidosis / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage* / Q000009:adverse effects; D000069286:Bortezomib / Q000008:administration & dosage* / Q000009:adverse effects; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000009:adverse effects; D003907:Dexamethasone / Q000008:administration & dosage / Q000009:adverse effects; D005260:Female; D006321:Heart / Q000187:drug effects / Q000503:physiopathology; D006333:Heart Failure / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality / Q000473:pathology; D006801:Humans; D007668:Kidney / Q000187:drug effects / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D012074:Remission Induction; D012189:Retrospective Studies; D016019:Survival Analysis; D013792:Thalidomide / Q000008:administration & dosage / Q000009:adverse effects; D016896:Treatment Outcome
chemical_list: D058227:Amyloidogenic Proteins; D013792:Thalidomide; D000069286:Bortezomib; D003907:Dexamethasone; D003520:Cyclophosphamide
keywords: AL amyloidosis; bortezomib; chemotherapy; novel agents