title: Outcome in renal Al amyloidosis after chemotherapy.
abstract: OBJECTIVE
  Chemotherapy in AL (primary or light chain) amyloidosis is associated with improved survival, but its effect on renal outcome has not been examined systematically. The purpose of this study was to evaluate the effect of chemotherapy on clinical outcome among patients with renal AL amyloidosis.
  
  
  METHODS
  We evaluated factors influencing survival among 923 patients with renal AL amyloidosis observed during a 21-year period, including 221 patients who became dialysis dependent. Factors associated with renal outcome were analyzed, including serum free light chain (FLC) response to chemotherapy using a simple subtraction formula applicable to all stages of chronic kidney disease. Patient survival and graft survival were analyzed in 21 renal transplantation recipients.
  
  
  RESULTS
  Median survival from diagnosis for the whole cohort was 35.2 months. Magnitude of FLC response with chemotherapy was strongly and independently associated with overall survival (P < .001) and renal outcome. Evaluable patients achieving more than 90% FLC response had a significantly higher rate of renal responses and lower rate of renal progression compared with patients achieving a 50% to 90% response, whose renal outcomes were, in turn, better than patients achieving less than 50% FLC response (P < .001). Median survival from dialysis dependence was 39.0 months, and median survival from renal transplantation was 89.0 months.
  
  
  CONCLUSIONS
  Renal outcome and overall outcome in AL amyloidosis are strongly associated with FLC response to chemotherapy and are best among patients achieving more than 90% suppression of the amyloidogenic monoclonal component. Survival on dialysis was substantially superior to that previously reported, and renal transplantation should be considered in selected patients with AL amyloidosis with end-stage renal disease.
authors.affiliation: National Amyloidosis Centre, Division of Medicine, University College London Medical School, Rowland Hill St, London NW3 2PF, United Kingdom.
authors.forename: Jennifer H
authors.initials: JH
authors.lastname: Pinney
authors.forename: Helen J
authors.initials: HJ
authors.lastname: Lachmann
authors.forename: Loveleen
authors.initials: L
authors.lastname: Bansi
authors.forename: Ashutosh D
authors.initials: AD
authors.lastname: Wechalekar
authors.forename: Janet A
authors.initials: JA
authors.lastname: Gilbertson
authors.forename: Dorota
authors.initials: D
authors.lastname: Rowczenio
authors.forename: Prayman T
authors.initials: PT
authors.lastname: Sattianayagam
authors.forename: Simon D J
authors.initials: SD
authors.lastname: Gibbs
authors.forename: Emanuela
authors.initials: E
authors.lastname: Orlandi
authors.forename: Nancy L
authors.initials: NL
authors.lastname: Wassef
authors.forename: Arthur R
authors.initials: AR
authors.lastname: Bradwell
authors.forename: Philip N
authors.initials: PN
authors.lastname: Hawkins
authors.forename: Julian D
authors.initials: JD
authors.lastname: Gillmore
mesh_terms: D000686:Amyloidosis / Q000150:complications / Q000188:drug therapy / Q000401:mortality*; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood*; D053208:Kaplan-Meier Estimate; D007674:Kidney Diseases / Q000150:complications / Q000188:drug therapy / Q000401:mortality*; D016030:Kidney Transplantation; D006435:Renal Dialysis; D016896:Treatment Outcome
chemical_list: D007147:Immunoglobulin Light Chains