title: Clinical characteristics and treatment outcome of children with acute lymphocytic leukemia and Down's syndrome. A Pediatric Oncology Group study.
abstract: Of 2947 children with acute lymphocytic leukemia (ALL), treated during three consecutive studies of the Pediatric Oncology Group (1974-1986), 52 (1.8%) had Down's Syndrome (DS). Comparison of clinical and laboratory characteristics showed no significant differences in leukocyte count, racial distribution, sex ratio, platelet count, incidence of mediastinal mass, lymphadenopathy or hepatosplenomegaly, or percentage of blood or bone marrow blasts for children with ALL with or without Down's Syndrome (DS-ALL or NDS-ALL, respectively). However, children with DS-ALL were slightly older at the time of presentation and had higher hemoglobin values. The relative frequency of each major immunophenotype (early pre-B, pre-B, T, or B) was also comparable for patients with or without DS. For this report, treatment regimens were categorized as either conventional (no consolidation therapy) or intensive. Cox regression analysis revealed that the presence of DS, a higher leukocyte count, black race, or age older than 10 years was independently associated with a poorer event-free survival (EFS) for children treated with conventional chemotherapy. However, for the cohort of children who received intensive chemotherapy, DS was no longer an independent risk factor. In fact, event-free survival (EFS) was markedly improved to a level comparable with that observed in the children diagnosed as having NDS-ALL. On the other hand, serious toxicity, requiring interruption of treatment, was significantly more frequent in the intensively treated children with DS compared with similarly treated patients with NDS-ALL, although deaths resulting from toxicity occurred infrequently.
authors.affiliation: Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia.
authors.forename: A H
authors.initials: AH
authors.lastname: Ragab
authors.forename: A
authors.initials: A
authors.lastname: Abdel-Mageed
authors.forename: J J
authors.initials: JJ
authors.lastname: Shuster
authors.forename: L S
authors.initials: LS
authors.lastname: Frankel
authors.forename: J
authors.initials: J
authors.lastname: Pullen
authors.forename: J
authors.initials: J
authors.lastname: van Eys
authors.forename: M P
authors.initials: MP
authors.lastname: Sullivan
authors.forename: J
authors.initials: J
authors.lastname: Boyett
authors.forename: M
authors.initials: M
authors.lastname: Borowitz
authors.forename: W M
authors.initials: WM
authors.lastname: Crist
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications*; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D016130:Immunophenotyping; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D011379:Prognosis; D012074:Remission Induction
chemical_list: D006454:Hemoglobins