title: [Efficacy of modified BFM-90 regimen on children and adolescents with T cell lymphoblastic lymphoma: a report of 20 cases].
abstract: OBJECTIVE
  T-cell lymphoblastic lymphoma in childhood and adolescence is an aggressive malignant disease with higher mortality. BFM-90 regimen for lymphoblastic lymphoma is one of the most effective regimens. This study was designed to evaluate efficacy and toxicities of modified BFM-90 regimen on Chinese children and adolescents with lymphoblastic lymphoma.
  
  
  METHODS
  A total of 20 naive children and adolescents with T cell lymphoblastic lymphoma were enrolled, 7 in stage III, and 13 in stage IV. Eighteen (90%) patients suffered from mediastinal mass with superior vena obstruction syndrome,10(50%) suffered from marrow invasion. All patients received modified BFM-90 regimen consisting of induction, consolidation and central nervous system prophylaxis, reinduced alleviation, and maintenance therapy. Total treatment duration was 2 years. Kaplan-Meier method was used to evaluate long-term survival rate.
  
  
  RESULTS
  After induction remission,18 patients (90%) achieved complete remission (CR), 1 had partial remission (PR), and 1 had progressive disease (PD), overall response rate was 95%. The 2 patients with PR or PD died of tumor progression. Of 2 patients at CR1 received APBSC, 1 relapsed after transplantation, but achieved CR and survived after salvage chemotherapy;1 survived all along. Of other patients achieved CR, 5 relapsed; of these 5 patients, 1 survived after allogeneic stem cell transplantation, 1 survived after autologous stem cell transplantation, 3 died of progressive disease after chemotherapy. The overall 3-year survival rate was 74%. All patients had myelosuppression of grade III-IV during the induction and reinduction phases, but the hemotologic toxicity was manageable.
  
  
  CONCLUSIONS
  Modified BFM-90 regimen is feasible for Chinese children and adolescent patients with lymphoblastic lymphoma, and may improve survival rate of these patients. The major side effect is myelosuppression, but it is manageable.
authors.affiliation: Department of Medical Oncology, Cancer Center, Sun Yat-sen University, Guangzhou, Guangdong 510060, P.R. China. gzsunxf@yahoo.com.cn
authors.forename: Xiao-Fei
authors.initials: XF
authors.lastname: Sun
authors.forename: Wen-Qi
authors.initials: WQ
authors.lastname: Jiang
authors.forename: Dong-Gen
authors.initials: DG
authors.lastname: Liu
authors.forename: Zhong-Jun
authors.initials: ZJ
authors.lastname: Xia
authors.forename: Hui-Qiang
authors.initials: HQ
authors.lastname: Huang
authors.forename: Li
authors.initials: L
authors.lastname: Zhang
authors.forename: Yu-Hong
authors.initials: YH
authors.lastname: Li
authors.forename: Zhong-Mei
authors.initials: ZM
authors.lastname: Zhou
authors.forename: Zi-Jun
authors.initials: ZJ
authors.lastname: Zhen
authors.forename: Yi
authors.initials: Y
authors.lastname: Xia
authors.forename: You-Jian
authors.initials: YJ
authors.lastname: He
authors.forename: Zhong-Zhen
authors.initials: ZZ
authors.lastname: Guan
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001215:Asparaginase / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D003561:Cytarabine / Q000627:therapeutic use; D003630:Daunorubicin / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D015122:Mercaptopurine / Q000627:therapeutic use; D008727:Methotrexate / Q000627:therapeutic use; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy* / Q000473:pathology / Q000628:therapy; D011241:Prednisone / Q000627:therapeutic use; D012074:Remission Induction; D033581:Stem Cell Transplantation; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D003561:Cytarabine; D014750:Vincristine; D003520:Cyclophosphamide; D015122:Mercaptopurine; D001215:Asparaginase; D011241:Prednisone; D008727:Methotrexate; D003630:Daunorubicin