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Leukemia (2009) 23, 11271130& 2009 Macmillan Publishers Limited All rights reserved 0887-6924/09 $32.00
http://www.nature.com/leu
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ORIGINAL ARTICLEEffective treatment of advanced-stage childhood lymphoblastic lymphoma without
prophylactic cranial irradiation: results of St Jude NHL13 study
JT Sandlund1, C-H Pui1, Y Zhou2, FG Behm3, M Onciu3, BI Razzouk1, N Hijiya1, D Campana1,3, MM Hudson1 and RC Ribeiro1
1Department of Oncology, St Jude Childrens Research Hospital, Memphis, TN, USA; 2Department of Biostatistics, St Jude Childrens Research Hospital, Memphis, TN, USA and 3Department of Pathology, St Jude Childrens Research Hospital, Memphis, TN, USA
There has been a steady improvement in cure rates for children with advanced-stage lymphoblastic non-Hodgkins lymphoma. To further improve cure rates whereas minimizing long-term toxicity, we designed a protocol (NHL13) based on a regimen for childhood T-cell acute lymphoblastic leukemia, which features intensive intrathecal chemotherapy for central nervous system-directed therapy and excludes prophylactic cranial irradiation. From 1992 to 2002, 41 patients with advanced-stage lymphoblastic lymphoma were enrolled on the protocol. Thirty patients had stage III and 11 had stage IV disease. Thirty-three cases had a precursor T-cell immunophenotype, ve had precursor B-cell immunophenotype and in three immunophenotype was not determined. Out of the 41 patients, 39 (95%) achieved a complete remission. The 5-year event-free rate was 82.96.3% (s.e.), and 5-year overall survival rate was 90.24.8% (median follow-up 9.3 years (range 4.6213.49 years)). Adverse events included two induction failures, one death from typhlitis during remission, three relapses and one secondary acute myeloid leukemia. The treatment described here produces high cure rates in children with lymphoblastic lymphoma without the use of prophylactic cranial irradiation.
Leukemia (2009) 23, 11271130; doi:http://dx.doi.org/10.1038/leu.2008.400
Web End =10.1038/leu.2008.400 ; published online 5 February 2009Keywords: advanced-stage; pediatric; lymphoblastic lymphoma
Introduction
The non-Hodgkins lymphomas (NHLs) of childhood are primarily high-grade tumors as dened by the National Cancer Institute Working Formulation.1,2 These comprise the Burkitt, large-cell and lymphoblastic subtypes as classied according to the World Health Organization system.2 Lymphoblastic lymphoma represents approximately 30% of all pediatric NHL. The vast majority is advanced-stage lymphoblastic lymphoma with a precursor T-cell immunophenotype.
The outcome of treatment for children with NHL has improved over the past 30 years, largely through sequential protocols that used increasingly intensive chemotherapy regimens guided by stage and histology or immunophenotype.310
With modern therapy, at least...