title: Long-term results of an ultra low-dose cytarabine-based regimen for the treatment of acute megakaryoblastic leukaemia in children with Down syndrome.
abstract: Children with acute megakaryoblastic leukaemia (AMKL) and Down syndrome (DS) show a favourable response to chemotherapy, probably due to increased sensitivity of the leukaemic blasts to cytarabine. In contrast, dose-intensive approaches have resulted in disproportionate treatment-related mortality in this group. The survival of children with AMKL and DS was retrospectively compared following treatment with a low-dose chemotherapy protocol, consisting of cytarabine (10 mg/m2/dose), retinylpalmitate and vincristine or standard chemotherapy. Event-free (67 +/- 11%) and overall survival (77 +/- 10%) at 5 years were not significantly different in both groups. Further reduction of treatment intensity in AMKL of children with DS, therefore, appears feasible.
authors.affiliation: Division of Hematology/Oncology, The Hospital for Sick Children, University of Toronto, Toronto, Ont., Canada.
authors.forename: Ali
authors.initials: A
authors.lastname: Al-Ahmari
authors.forename: Niketa
authors.initials: N
authors.lastname: Shah
authors.forename: Lillian
authors.initials: L
authors.lastname: Sung
authors.forename: Alvin
authors.initials: A
authors.lastname: Zipursky
authors.forename: Johann
authors.initials: J
authors.lastname: Hitzler
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D003561:Cytarabine / Q000008:administration & dosage; D004224:Diterpenes; D004314:Down Syndrome / Q000188:drug therapy*; D004334:Drug Administration Schedule; D004812:Epidemiologic Methods; D006801:Humans; D007947:Leukemia, Megakaryoblastic, Acute / Q000188:drug therapy*; D000084562:Retinyl Esters; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage; D014801:Vitamin A / Q000008:administration & dosage / Q000031:analogs & derivatives
chemical_list: D004224:Diterpenes; D000084562:Retinyl Esters; D003561:Cytarabine; D014801:Vitamin A; C014794:retinol palmitate; D014750:Vincristine