title: Clinical Characteristics and Treatment Outcome of Chinese Patients With Systemic Amyloid Light-Chain Amyloidosis: A Retrospective Single-Center Analysis.
abstract: BACKGROUND
  Amyloid light-chain (AL) amyloidosis is a disease caused by tissue deposition of light-chain proteins in vital organs that results in progressive organ damage. We analyzed the clinical characteristics of 123 AL amyloidosis patients and performed an overall survival (OS) analysis to identify critical baseline factors.
  
  
  METHODS
  Patients (median age, 54 years) were diagnosed with organ involvement of kidney (98.4%), gastrointestinal (73%), cardiac (56%), liver (13%), or nervous system (10%), and multiorgan involvement was observed in 91% of patients. Treatment regimens of transplantation, bortezomib plus dexamethasone, melphalan plus dexamethasone, and prednisone-based regimens or no treatment resulted in 3-year OS rates of 72%, 60%, 55%, and 41%, respectively.
  
  
  RESULTS
  Median OS was 38 months and was affected by age (≥ 65 years), hypoalbuminemia, renal failure, heart involvement, and organ response time (within 3 months). Multivariate analysis indicated that these were independent prognostic factors on OS except for age.
  
  
  CONCLUSIONS
  The AL amyloidosis patients in this study presented somewhat different features and outcomes compared to others, with younger age and higher rates of organ involvement.
authors.affiliation: Department of Hematology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
authors.forename: Qian
authors.initials: Q
authors.lastname: Zhao
authors.affiliation: Department of Hematology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
authors.forename: Feng
authors.initials: F
authors.lastname: Li
authors.affiliation: Department of Hematology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
authors.forename: Ping
authors.initials: P
authors.lastname: Song
authors.affiliation: Department of Hematology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
authors.forename: Xiaogang
authors.initials: X
authors.lastname: Zhou
authors.affiliation: Department of Hematology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
authors.forename: Liping
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Hematology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
authors.forename: Yaping
authors.initials: Y
authors.lastname: Yu
authors.affiliation: Department of Hematology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
authors.forename: Zhiming
authors.initials: Z
authors.lastname: An
authors.affiliation: Department of Hematology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
authors.forename: Xuli
authors.initials: X
authors.lastname: Wang
authors.affiliation: Department of Hematology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China. Electronic address: zhaiyongping66@163.com.
authors.forename: Yongping
authors.initials: Y
authors.lastname: Zhai
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000188:drug therapy* / Q000378:metabolism*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D044466:Asian People; D000069286:Bortezomib / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D006801:Humans; D008297:Male; D008558:Melphalan / Q000627:therapeutic use; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D019172:Transplantation Conditioning / Q000379:methods; D016896:Treatment Outcome
chemical_list: D000682:Amyloid; D000069286:Bortezomib; D003907:Dexamethasone; D008558:Melphalan; D011241:Prednisone
keywords: Bortezomib; HDM/SCT; Melphalan; Overall survival; Treatment response