title: Characteristics and long-term outcome of patients with systemic immunoglobulin light-chain amyloidosis.
abstract: OBJECTIVE
  Immunoglobulin light-chain (AL) amyloidosis is a systemic disorder that causes progressive organ dysfunction. The optimal treatment strategy requires accurate patient stratification with an emphasis on the extent of cardiac involvement. Reports on its prognosis are sparse and predominantly originate from highly selected centers. We aimed to evaluate patient characteristics and outcomes in a cohort treated at a single center.
  
  
  METHODS
  This is a single-center retrospective study of 63 consecutive patients diagnosed with AL amyloidosis between January 2000 and December 2012. Patients were evaluated by treatment strategy and cardiac involvement.
  
  
  RESULTS
  The mean age at diagnosis was 61.4 years (±8.9), and 39 patients (62%) were male. Thirty-two (51%) patients presented with cardiac amyloid involvement (CA) and the remaining 31 (49%) had noncardiac amyloidosis (NCA). The median follow-up time was 12.7 months (0.3-90.8), and 38 (60%) patients died during follow-up. The median overall survival (OS) was 29 months (95% CI 12.1-57.2) and the OS was not significantly lower for patients with CA compared to NCA (log-rank = 0.21).
  
  
  CONCLUSIONS
  The prognosis in AL amyloidosis is grave, but the outcome with treatment in the current series was comparable to those in series from larger centers. CA did not significantly predict the OS.
authors.affiliation: Department of Cardiology, The Heart Centre, Copenhagen, Denmark.
authors.forename: Lærke Marie
authors.initials: LM
authors.lastname: Nelson
authors.forename: Finn
authors.initials: F
authors.lastname: Gustafsson
authors.forename: Peter
authors.initials: P
authors.lastname: Gimsing
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D003710:Demography; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D008297:Male; D008558:Melphalan / Q000627:therapeutic use; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism; D012189:Retrospective Studies; D015996:Survival Rate; D014182:Transplantation, Autologous
chemical_list: D018906:Antineoplastic Agents, Alkylating; D007147:Immunoglobulin Light Chains; D008558:Melphalan