title: Treatment with all-trans retinoic acid and anthracycline monochemotherapy for children with acute promyelocytic leukemia: a multicenter study by the PETHEMA Group.
abstract: OBJECTIVE
  To analyze the simultaneous combination of all-trans retinoic acid (ATRA) and anthracycline monochemotherapy for children with acute promyelocytic leukemia (APL).
  
  
  METHODS
  Since November 1996, 66 children (younger than 18 years) with genetically proven APL received induction therapy with ATRA and idarubicin. Consolidation therapy consisted of three courses of anthracycline monochemotherapy. After November 1999, patients with intermediate and high risk of relapse received consolidation therapy with ATRA and slightly reinforced doses of idarubicin. Maintenance therapy consisted of ATRA and low-dose mercaptopurine and methotrexate.
  
  
  RESULTS
  Thirty-nine girls (59%) and 27 boys (41%) were included in this study. The WBC count at presentation was more than 10 x 10(9)/L in 26 patients (39%). Sixty-one children (92%) achieved complete remission (CR). Early deaths from hemorrhage and retinoic acid syndrome occurred in three patients and two patients, respectively. Toxicity was manageable during consolidation and maintenance therapy. No deaths in CR, clinical cardiomyotoxicity, or secondary malignancy occurred. Two patients had molecular persistence at the end of consolidation. Three clinical relapses and two molecular relapses were also observed. Apart from one molecular relapse, all these events occurred among children with hyperleukocytosis. The 5-year cumulative incidence of relapse was 17%, whereas disease-free and overall survival rates were 82% and 87%, respectively.
  
  
  CONCLUSIONS
  A high incidence of hyperleukocytosis in children with APL was confirmed. Besides low toxicity and a high degree of compliance, a risk-adapted therapy combining ATRA and anthracycline monochemotherapy showed an antileukemic efficacy comparable to those previously reported with other chemotherapy combinations in children.
authors.affiliation: Servicio de Hematología, Hospital Universitario La Fe, Avenida Campanar 21, 46009 Valencia, Spain.
authors.forename: Juan J
authors.initials: JJ
authors.lastname: Ortega
authors.forename: Luis
authors.initials: L
authors.lastname: Madero
authors.forename: Guillermo
authors.initials: G
authors.lastname: Martín
authors.forename: Amparo
authors.initials: A
authors.lastname: Verdeguer
authors.forename: Purificación
authors.initials: P
authors.lastname: García
authors.forename: Ricardo
authors.initials: R
authors.lastname: Parody
authors.forename: José
authors.initials: J
authors.lastname: Fuster
authors.forename: Antonio
authors.initials: A
authors.lastname: Molines
authors.forename: Andrés
authors.initials: A
authors.lastname: Novo
authors.forename: Guillermo
authors.initials: G
authors.lastname: Debén
authors.forename: Antonia
authors.initials: A
authors.lastname: Rodríguez
authors.forename: Eulogio
authors.initials: E
authors.lastname: Conde
authors.forename: Javier
authors.initials: J
authors.lastname: de la Serna
authors.forename: María J
authors.initials: MJ
authors.lastname: Allegue
authors.forename: Francisco J
authors.initials: FJ
authors.lastname: Capote
authors.forename: José D
authors.initials: JD
authors.lastname: González
authors.forename: Pascual
authors.initials: P
authors.lastname: Bolufer
authors.forename: Marcos
authors.initials: M
authors.lastname: González
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Sanz
mesh_terms: D000293:Adolescent; D018943:Anthracyclines / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015255:Idarubicin / Q000008:administration & dosage; D015994:Incidence; D015473:Leukemia, Promyelocytic, Acute / Q000188:drug therapy* / Q000453:epidemiology; D008297:Male; D015122:Mercaptopurine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy*; D011379:Prognosis; D012074:Remission Induction; D012307:Risk Factors; D015996:Survival Rate; D014212:Tretinoin / Q000008:administration & dosage
chemical_list: D018943:Anthracyclines; D014212:Tretinoin; D015122:Mercaptopurine; D008727:Methotrexate; D015255:Idarubicin