title: Relapsed or refractory anaplastic large-cell lymphoma in children and adolescents after Berlin-Frankfurt-Muenster (BFM)-type first-line therapy: a BFM-group study.
abstract: OBJECTIVE
  To evaluate risk factors for outcome in children and adolescents with relapse of anaplastic large-cell lymphoma (ALCL) after comparable first-line therapy.
  
  
  METHODS
  We analyzed a population-based cohort of 74 children with relapsed ALCL after Berlin-Frankfurt-Muenster-type first-line therapy between April 1990 and December 2003. The recommended salvage strategy was reinduction chemotherapy followed by autologous hematopoietic stem-cell transplantation (SCT).
  
  
  RESULTS
  With a median follow-up time of 8.4 years (range, 4.5 to 16.4 years), the 5-year overall survival (OS) rate after first relapse was 57% ± 6%. Survival correlated with time of relapse and clinically advanced dissemination. Five-year OS of 16 patients who experienced progression during first-line therapy was 25% ± 11% compared with 66% ± 6% for 58 patients with a later relapse (P = .002). Five-year OS of 11 patients with bone marrow or CNS involvement was 27% ± 13% compared with 62% ± 6% for 63 patients without involvement (P = .001). Five-year event-free survival (EFS) and OS of 39 children who received the recommended autologous SCT were 59% ± 8% and 77% ± 7%, respectively. EFS after autologous SCT was significantly associated with time to relapse (progression: n = 3; EFS, 0; later relapse: n = 36; EFS, 64% ± 8%; P = .014) and CD3 expression (CD3 negative: n = 25; EFS, 72% ± 9%; CD3 positive: n = 11; EFS, 18% ± 12%; P < .001), but not with site of relapse, conditioning regimen, or graft manipulation. No relapses occurred among 10 patients with relapsed CD3-positive ALCL treated with allogeneic SCT.
  
  
  CONCLUSIONS
  Reinduction chemotherapy followed by autologous SCT proved feasible and efficacious for patients with a first relapse of CD3-negative ALCL after first-line therapy. Patients with progression during first-line therapy or relapsed CD3-positive ALCL may benefit from allogeneic SCT.
authors.affiliation: NHL-BFM Study Center and Department of Pediatric Hematology and Oncology, Justus-Liebig-University, D-35392 Giessen, Germany. Wilhelm.woessmann@paediat.med.uni-giessen.de
authors.forename: Willi
authors.initials: W
authors.lastname: Woessmann
authors.forename: Martin
authors.initials: M
authors.lastname: Zimmermann
authors.forename: Meike
authors.initials: M
authors.lastname: Lenhard
authors.forename: Birgit
authors.initials: B
authors.lastname: Burkhardt
authors.forename: Claudia
authors.initials: C
authors.lastname: Rossig
authors.forename: Bernhard
authors.initials: B
authors.lastname: Kremens
authors.forename: Peter
authors.initials: P
authors.lastname: Lang
authors.forename: Andishe
authors.initials: A
authors.lastname: Attarbaschi
authors.forename: Georg
authors.initials: G
authors.lastname: Mann
authors.forename: Ilske
authors.initials: I
authors.lastname: Oschlies
authors.forename: Wolfram
authors.initials: W
authors.lastname: Klapper
authors.forename: Alfred
authors.initials: A
authors.lastname: Reiter
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005240:Feasibility Studies; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017728:Lymphoma, Large-Cell, Anaplastic / Q000188:drug therapy* / Q000601:surgery*; D008297:Male; D011237:Predictive Value of Tests; D011379:Prognosis; D012008:Recurrence; D012074:Remission Induction; D018570:Risk Assessment; D012307:Risk Factors; D016879:Salvage Therapy / Q000379:methods; D014182:Transplantation, Autologous; D016896:Treatment Outcome