title: Bortezomib with dexamethasone as first-line treatment for AL amyloidosis with renal involvement.
abstract: Although bortezomib has reported efficacy in light chain (AL) amyloidosis, the role of bortezomib in combination with dexamethasone (BD) as the first-line treatment for patients with AL amyloidosis has not been determined. We analyzed the outcomes of 72 consecutive unselected patients, which received primary therapy with BD in a single center. The patients were newly diagnosed with AL amyloidosis with renal (100%), cardiac (72%), hepatic (19%) or nervous system (10%) involvement and underwent a median of 2 (1-6) cycles of BD treatment. A hematologic response was achieved in 75% of the patients within a median period of 2 months, and 45% of those patients achieved a complete response. A renal response was achieved in 50% and 60% of patients at 1 year and 2 years, respectively, and a cardiac response was achieved in 40% and 46% of patients at 1 year and 2 years, respectively. After a median follow-up period of 24 months, the median duration of progression free survival was 45 months, and the estimated overall survival rates at 12 and 24 months were 83% and 76%, respectively. Baseline Eastern Cooperative Oncology Group performance status and proteinuria were associated with overall survival. The BD regimen induced high rates of rapid hematologic and organ responses in AL amyloidosis patients.
authors: a National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine , Nanjing , China.
authors: Xianghua
authors: X
authors: Huang
authors: a National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine , Nanjing , China.
authors: Qingwen
authors: Q
authors: Wang
authors: a National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine , Nanjing , China.
authors: Wencui
authors: W
authors: Chen
authors: a National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine , Nanjing , China.
authors: Guisheng
authors: G
authors: Ren
authors: a National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine , Nanjing , China.
authors: Zhihong
authors: Z
authors: Liu
mesh_terms: D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000188:drug therapy* / Q000401:mortality; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D000069286:Bortezomib / Q000627:therapeutic use*; D003907:Dexamethasone / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis; D053208:Kaplan-Meier Estimate; D007674:Kidney Diseases / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D061988:Proteasome Inhibitors / Q000627:therapeutic use*; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D061988:Proteasome Inhibitors; D000069286:Bortezomib; D003907:Dexamethasone
keywords: AL amyloidosis; bortezomib; dexamethasone; efficacy; safety