Excellent outcome of acute lymphoblastic leukaemia with TCF3-PBX1 rearrangement in Hong Kong

Pediatr Blood Cancer. 2018 Dec;65(12):e27346. doi: 10.1002/pbc.27346. Epub 2018 Jul 26.


Objective: The aim of this study was to review clinical outcomes and prognosis of paediatric patients with acute lymphoblastic leukaemia (ALL) with TCF3-PBX1 rearrangement.

Patients: All children in Hong Kong diagnosed with ALL with TCF3-PBX1 rearrangement over the past two decades were included.

Methods: Six hundred and twenty-four newly diagnosed patients with ALL from four consecutive studies were enrolled from 1997 to 2016. Patients carrying TCF3-PBX1 rearrangement and patients at intermediate risk without the gene expression were compared for clinical characteristics, overall survival and event-free survival (EFS).

Results: The TCF3-PBX1 rearrangement was detected in 30 of 624 patients (4.8%). Results were consistent across the consecutive clinical trials employed in the past two decades. Compared with 239 intermediate risk patients without TCF3-PBX1 rearrangement, the 5-year overall survival and EFS for patients with TCF3-PBX1 rearrangement was superior, with both at 100% (P = 0.12 and P = 0.029).

Conclusion: This population-based study over the past 20 years demonstrated that patients with TCF3-PBX1 rearrangement had favourable EFS compared with other intermediate risk patients treated with a similar chemotherapy backbone.

Keywords: EFS; TCF3-PBX1; acute lymphoblastic leukaemia; survival; t(1;19).

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Gene Rearrangement*
  • Hong Kong
  • Humans
  • Infant
  • Male
  • Oncogene Proteins, Fusion / genetics*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / drug therapy
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / genetics
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / mortality
  • Retrospective Studies
  • Survival Rate


  • Oncogene Proteins, Fusion
  • TCF3-PBX1 fusion protein, human