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Chinese Journal of Evidence-Based Pediatrics ›› 2022, Vol. 17 ›› Issue (2): 98-103.

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Clinical biological characteristics and prognostic significance of pediatric T cell acute lymphoblastic leukemia: A retrospective cohort study

HU Hairui1, YUAN Yuan1, LI Jun2, XUE Tianlin2, WANG Ying2, LIU Shuguang2, GAO Chao2, ZHENG Huyong1    

  1. 1 Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics, Capital Medical University; Key Laboratory of Major Diseases in Children, Ministry of Education; Hematology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China; 2 Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics, Capital Medical University; Key Laboratory of Major Diseases in Children, Ministry of Education; Laboratory of Hematologic Diseases, Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China
  • Received:2021-03-31 Revised:2022-04-07 Online:2022-04-25 Published:2022-04-25
  • Contact: GAO Chao, email: gaochaoconnie@live.cn; ZHENG Huyong, email: zhenghuyong@vip.sina.com

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Abstract: Background：Currently, there are differences in the outcomes of risk-stratified treatment in pediatric acute T lymphocytic leukemia (T-ALL). Objective：To explore the association between biological characteristics and clinical features, early treatment responses and longterm outcomes of pediatric T-ALL. Design：Retrospective cohort study. Methods：The immunophenotypic markers and fusion genes at diagnosis and its association with clinical features, early treatment response and long-term outcomes of T-ALL patients treated with CCLG-ALL-2008 protocol in Beijing Children's Hospital affiliated to Capital Medical University from March 2008 to September 2018 were retrospectively analyzed. Main outcome measures：Event free survival (EFS) and overall survival (OS). Results：A total of 101 children with T-ALL were enrolled, including 76 males (75.25%). SIL-TAL1 was the most common fusion gene (21.8%, 22/101) and the proportion of patients classified into high-risk of SIL-TAL1+ was significantly higher than that of SIL-TAL1-. The expression rate of stem cell marker CD34 was 45.5% (46/101). The expression of CD2 was associated with high peripheral white blood cell; the expression of CD34 was associated with poor prednisone response and high minimal residual disease (MRD) at day 15; the expression of CD33 was associated with morphological non-remission and high MRD at the end of induction; while the expression of CD10 was associated with good prednisone response. By K-M analysis, positive SIL-TAL1, poor response to prednisone treatment, non-remission of bone marrow morphology on day 15, high MRD at day 33 and classification of high-risk were associated with adverse EFS and OS (P＜0.05 respectively) . Furthermore, MRD≥1% at day 33 and SIL-TAL1 positive were also validated as independent prognostic factors for EFS and OS. The hazard ratios of MRD ≥1% at day 33 for EFS was 1.96 (95%CI: 1.114-3.452, P=0.020) and for OS was 2.062 (95%CI: 1.138-3.734, P=0.017); SIL-TAL1+ for EFS was 2.536 (95%CI: 1.053-6.104, P=0.038) and for OS was 2.921 (95%CI: 1.144-7.457, P=0.025). Conclusion：T-ALL is a group of heterogeneous diseases. SIL-TAL1 and early MRD can predict the long-term prognosis on the MRD based treatment protocol.

Key words: T-cell acute lymphoblastic leukemia, Children, Prognosis, SIL-TAL1

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