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Outcomes of light‐chain amyloidosis patients treated with first‐line bortezomib: a collaborative retrospective multicenter assessment
Authors:Moshe E. Gatt  Izhar Hardan  Evgeni Chubar  Celia Suriu  Tamar Tadmor  Olga Shevetz  Paulina Patachenco  Najib Dally  Shay Yeganeh  Mouna Ballan‐Haj  Yael Cohen  Svetlana Trestman  Eli Muchtar  Hila Magen  Julia Jakubinsky  Irit Avivi  the Israeli MM study group
Affiliation:1. Department of Hematology, Hadassah Medical Center, Jerusalem, Israel;2. Hematology Unit, Meir Medical Center, Kfar Saba, Israel;3. Department of Hematology, HaEmek Medical Center, Afula, Israel;4. Department of Hematology, Western Galilee Hospital, Nahariya, Israel;5. Hematology Unit, Bnai‐Zion Medical Center, Haifa, Israel;6. Department of Hematology, Kaplan Medical Center, Rehovot, Israel;7. Department of Hematology, Wolfson Medical Center, Holon, Israel;8. Department of Hematology, Ziv Medical Center, Safed, Israel;9. Hematology Unit, Poria Medical Center, Tiberias, Israel;10. Hematology Unit, Carmel Medical Center, Haifa, Israel;11. Department of Hematology, Sorasky Medical Center, Tel Aviv, Israel;12. Department of Hematology, Rabin Medical Center, Petah Tikva, Israel;13. Department of Hematology, Rambam Health Care Campus, Haifa, Israel
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.
Keywords:AL amyloidosis  novel agents  chemotherapy  bortezomib
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