title: Outcome of children with high-risk acute lymphoblastic leukemia (HR-ALL): Nordic results on an intensive regimen with restricted central nervous system irradiation.
abstract: BACKGROUND
  Improvement in outcome of childhood high-risk (HR) ALL was sought with a very intensive Nordic protocol leaving most patients without CNS-RT.
  
  
  METHODS
  A total of 426 consecutive children entered the NOPHO-92 HR-ALL program. HR criteria included WBC > or =50 x 10(9)/L, CNS or testicular involvement, T-cell, lymphomatous features, t(9;22), t(4;11), or slow response. Of these, 152 children had very high risk (VHR) with special definitions. CNS consolidation was based on high-dose MTX (8 g/m2) and ARA-C (12 g/m2) alternating. VHR patients also received cranial RT.
  
  
  RESULTS
  The 9-year EFS was 61 +/- 3%, OS 74 +/- 2%, and EFS for T-ALL 62 +/- 4%. Cumulative incidence of isolated CNS relapse was 4.7 +/- 1%, and CNS relapse in total 9.9 +/- 2%. Poor prognostic factors were WBC > or =200 x 10(9)/L and a very slow response.
  
  
  CONCLUSIONS
  HR-ALL was successfully treated on the NOPHO-92 regimen, with a relatively low CNS relapse rate for non-irradiated children. WBC > or =200 x 10(9)/L and very slow response emerged as strong poor prognostic factors.
authors.affiliation: Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland. ulla.pihkala@hus.fi
authors.forename: Ulla M
authors.initials: UM
authors.lastname: Saarinen-Pihkala
authors.forename: G
authors.initials: G
authors.lastname: Gustafsson
authors.forename: N
authors.initials: N
authors.lastname: Carlsen
authors.forename: T
authors.initials: T
authors.lastname: Flaegstad
authors.forename: E
authors.initials: E
authors.lastname: Forestier
authors.forename: A
authors.initials: A
authors.lastname: Glomstein
authors.forename: J
authors.initials: J
authors.lastname: Kristinsson
authors.forename: M
authors.initials: M
authors.lastname: Lanning
authors.forename: H
authors.initials: H
authors.lastname: Schroeder
authors.forename: L
authors.initials: L
authors.lastname: Mellander
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D016543:Central Nervous System Neoplasms / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D003561:Cytarabine / Q000008:administration & dosage; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000628:therapy*; D012307:Risk Factors; D033581:Stem Cell Transplantation; D014184:Transplantation, Homologous; D016896:Treatment Outcome
chemical_list: D003561:Cytarabine; D008727:Methotrexate