title: [Prognostic factors in light chain amyloidosis].
abstract: BACKGROUND
  Light chain amyloidosis is characterized by extracellular deposition of a fibrillar material derived from immunglobulin light chain fragments.
  
  
  OBJECTIVE
  The aim of the authors was to assess survival depending on cardiac involvement, therapy, and presence of myeloma.
  
  
  METHODS
  The authors studied a retrospective cohort of 29 patients with light chain amyloidosis (13 kappa, 16 lambda) treated in their institution between 2005 and 2014.
  
  
  RESULTS
  Twenty-one patients had primary amyloidosis, while 8 had coexisting multiple myeloma. One, two and three or more organs were involved in 4, 8, and 17 patients, respectively. Cardiac involvement (22 cases) inversely correlated with survival. Fifteen (52%) patients received chemotherapy only, while 14 (48%) underwent autologous stem cell transplantation with a median survival of 87 and 11.4 months, respectively. Two patients had heart transplantation and survived 70 and 30 months. Median overall survival was 75.8 months.
  
  
  CONCLUSIONS
  Cardiac transplantation followed by autologous stem cell transplantation is feasible in selected patients with light chain amyloidosis and heart failure.
authors.affiliation: Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.
authors.forename: Nóra Rebeka
authors.initials: NR
authors.lastname: Jávorniczky
authors.affiliation: Emory University School of Medicine Atlanta, USA.
authors.forename: Imre
authors.initials: I
authors.lastname: Bodó
authors.affiliation: Egyesített Szent István és Szent László Kórház Budapest.
authors.forename: Tamás
authors.initials: T
authors.lastname: Masszi
authors.affiliation: Egyesített Szent István és Szent László Kórház Budapest.
authors.forename: Gábor
authors.initials: G
authors.lastname: Mikala
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000150:complications* / Q000276:immunology / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis; D007145:Immunoglobulin kappa-Chains / Q000378:metabolism; D007146:Immunoglobulin lambda-Chains / Q000378:metabolism; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications / Q000188:drug therapy / Q000401:mortality / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D033581:Stem Cell Transplantation; D014182:Transplantation, Autologous; D016896:Treatment Outcome
chemical_list: D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains
keywords: amyloidosis; autologous stem cell transplantation; autológ őssejtátültetés; bortezomib; könnyűlánc; light chain