title: Increased post-induction intensification improves outcome in children and adolescents with a markedly elevated white blood cell count (≥200 × 10(9) /l) with T cell acute lymphoblastic leukaemia but not B cell disease: a report from the Children's Oncology Group.
abstract: Children and adolescents presenting with a markedly elevated white blood cell (ME WBC) count (WBC ≥200 × 10(9) /l) comprise a unique subset of high-risk patients with acute lymphoblastic leukaemia (ALL). We evaluated the outcomes of the 251 patients (12% of the study population) with ME WBC treated on the Children's Cancer Group-1961 protocol. Patients were evaluated for early response to treatment by bone marrow morphology; those with a rapid early response were randomized to treatment regimens testing longer and stronger post-induction therapy. We found that ME WBC patients have a poorer outcome compared to those patients presenting with a WBC <200 × 10(9) /l (5-year event-free survival 62% vs. 73%, P = 0·0005). Longer duration of therapy worsened outcome for T cell ME WBC with a trend to poorer outcome in B-ALL ME WBC patients. Augmented therapy benefits T cell ME WBC patients, similar to the entire study cohort, however, there appeared to be no impact on survival for B-ALL ME WBC patients. ME WBC was not a prognostic factor for T cell patients. In patients with high risk features, B lineage disease in association with ME WBC has a negative impact on survival.
authors.affiliation: Department of Hematology Oncology, Children's Hospital & Research Center Oakland, Oakland, CA, USA.
authors.forename: Caroline
authors.initials: C
authors.lastname: Hastings
authors.forename: Paul S
authors.initials: PS
authors.lastname: Gaynon
authors.forename: James B
authors.initials: JB
authors.lastname: Nachman
authors.forename: Harland N
authors.initials: HN
authors.lastname: Sather
authors.forename: Xiaomin
authors.initials: X
authors.lastname: Lu
authors.forename: Meenakshi
authors.initials: M
authors.lastname: Devidas
authors.forename: Nita L
authors.initials: NL
authors.lastname: Seibel
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D001215:Asparaginase / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D016371:Cranial Irradiation; D003520:Cyclophosphamide / Q000008:administration & dosage; D003630:Daunorubicin / Q000008:administration & dosage; D018572:Disease-Free Survival; D004305:Dose-Response Relationship, Drug; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015255:Idarubicin / Q000008:administration & dosage; D007223:Infant; D053208:Kaplan-Meier Estimate; D015448:Leukemia, B-Cell / Q000097:blood / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy; D007958:Leukocyte Count; D007964:Leukocytosis / Q000209:etiology*; D008297:Male; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000097:blood / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy; D011241:Prednisone / Q000008:administration & dosage; D012074:Remission Induction; D012306:Risk; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D001215:Asparaginase; D011241:Prednisone; D015255:Idarubicin; D003630:Daunorubicin
keywords: acute lymphoblastic leukaemia; elevated white blood cell count; intensification; paediatric