title: [Clinical Characteristics and Treatment Efficacy of Children with SIL/TAL1 Positive T-Cell Acute Lymphoblastic Leukemia].
abstract: OBJECTIVE
  To investigate the clinical features, treatment and prognosis of children with SIL-TAL1 fusion gene positive T-cell acute lymphoblastic leukemia (T-ALL).
  
  
  METHODS
  The data of 101 children with T-ALL were collected from April 2005 to November 2012 in Beijing Children's Hospital. The common clinical features, early treatment response, minimal residual disease (MRD), event-free survival (EFS) and relapse-free survival (RFS) were compared between children with SIL-TAL1 positive and negative T-ALL. The treatment efficacy in children with SIL-TAL1 positive T-ALL was compared between BCH-2003 and CCLG-2008 protocol.
  
  
  RESULTS
  Out of 101 cases, 22 cases (21.9%) of T-ALL carried SIL-TAL1 fusion gene. The distribution of sex, age, response to prednisone and central nervous system (CNS) involvement were no significant different at diagnosis between SIL-TAL1 positive and negetive patients. However, the WBC count in SIL-TAL1 positive cases were significantly higher than that of SIL-TAL1 negative cases at diagnosis (P<0.05). Additionally, MRD levels were not significantly different between children with SIL-TAL1 positive or negative T-ALL at 3 time points including: after the remission induction therapy, before delayed intensive therapy II and before maintenance therapy. However, the number of cases with high MRD levels before consolidation therapy were more in SIL-TAL1 positive group than that in SIL-TAL1 negative group (P<0.05). The cases with high MRD levels before delayed intensive therapy I was more in SIL-TAL1 negative group than that in the SIL-TAL1 positive group (P>0.05). Besides, there were no significant differences in 5-year EFS and RFS between the two groups. The risk of 22 children with SIL-TAL1 positive acute T-ALL was again stratified according to the typing stemdard in CCLG-2008 protocol, as a result, the risk in BCH-2003 group (10 cases) was significantly higher than that in BCH-2008 group (12 cases) (P<0.05), but no significant difference was found in common clinical features, early treatment response, MRD levels and treatment efficacy.
  
  
  CONCLUSIONS
  Although WBC level was significantly higher in SIL-TAL1 positive group than that in SIL-TAL1 negative group, the treatment efficacy in SIL-TAL1 positive group was similar to SIL-TAL1 negative group. Meanwhile, the children with SIL-TAL1+ T-ALL may respond poorly to early intensive therapy, the BCH-2003 protocol may be more suitable for the patients with this subtype of leukemia.
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Xiao
authors.initials: X
authors.lastname: Liu
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Wei-Jing
authors.initials: WJ
authors.lastname: Li
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Xiao-Xi
authors.initials: XX
authors.lastname: Zhao
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Chao
authors.initials: C
authors.lastname: Gao
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Zhao
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Jin
authors.initials: J
authors.lastname: Jiang
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Rui-Dong
authors.initials: RD
authors.lastname: Zhang
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Xie
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Hui-Wen
authors.initials: HW
authors.lastname: Shi
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Bin
authors.initials: B
authors.lastname: Wang
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Yong-Hong
authors.initials: YH
authors.lastname: Zhang
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Xiao-Li
authors.initials: XL
authors.lastname: Ma
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Xuan
authors.initials: X
authors.lastname: Zhou
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
authors.forename: Min-Yuan
authors.initials: MY
authors.lastname: Wu
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China. E-mail: cuileilsh@163.co.
authors.forename: Lei
authors.initials: L
authors.lastname: Cui
authors.affiliation: Hematologic Oncology Center, Beijing Key Laboratory of Pediatric Hematology and Oncology; Key Laboratory of Major Diseases in Children, Ministry of Education; National Key Discipline of Pediatrics; Beijing Children's Hospital, Capital Medical University, Beijing 100045, China. E-mail: ericlzg70@ hotmail.com.
authors.forename: Zhi-Gang
authors.initials: ZG
authors.lastname: Li
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols; D002648:Child; D018572:Disease-Free Survival; D006801:Humans; D018365:Neoplasm, Residual / Q000473:pathology; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D011379:Prognosis; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D015514:Oncogene Proteins, Fusion; C547584:SIL-TAL1 fusion protein, human