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In Ovo Xenografting of Patient-Derived Acute Lymphoblastic Leukemia (ALL) Cells (PDX-ALL)
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In Ovo Xenografting of Patient-Derived Acute Lymphoblastic Leukemia (ALL) Cells (PDX-ALL)

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

Anna Lin1, Frankie W T Cheng1, Alan K S Chiang2

  • 1Department of Paediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong, China.

Pediatric Blood & Cancer
|July 28, 2018
PubMed

Insights

Pediatric acute lymphoblastic leukemia (ALL) patients with TCF3-PBX1 rearrangement show superior event-free survival. This finding highlights the favorable prognosis associated with this genetic marker in childhood ALL.

Area of Science:

  • Pediatric Oncology
  • Hematology
  • Genetics

Background:

  • Acute lymphoblastic leukemia (ALL) is a common childhood cancer.
  • Genetic rearrangements play a crucial role in ALL prognosis.
  • The TCF3-PBX1 rearrangement is a specific genetic marker in ALL.

Purpose of the Study:

  • To review clinical outcomes and prognosis of pediatric ALL patients with TCF3-PBX1 rearrangement.
  • To compare survival rates of TCF3-PBX1 rearranged ALL with intermediate-risk ALL without the rearrangement.
  • To assess the impact of TCF3-PBX1 rearrangement on event-free survival (EFS) and overall survival.

Main Methods:

  • Retrospective analysis of pediatric ALL patients diagnosed in Hong Kong over two decades (1997-2016).
  • Inclusion of 624 newly diagnosed ALL patients across four consecutive studies.
  • Comparison of clinical characteristics, overall survival, and EFS between TCF3-PBX1 rearranged patients and intermediate-risk patients without the rearrangement.

Main Results:

  • TCF3-PBX1 rearrangement was identified in 4.8% (30/624) of patients.
  • Patients with TCF3-PBX1 rearrangement demonstrated superior 5-year overall survival and EFS (100%) compared to intermediate-risk patients without the rearrangement.
  • Results were consistent across multiple clinical trials over the study period.

Conclusions:

  • Pediatric ALL patients with TCF3-PBX1 rearrangement exhibit a favorable prognosis.
  • The TCF3-PBX1 rearrangement is associated with improved event-free survival compared to other intermediate-risk ALL patients.
  • This population-based study confirms the favorable EFS for TCF3-PBX1 rearranged ALL treated with standard chemotherapy.
Abstract

Keywords:
EFSTCF3-PBX1acute lymphoblastic leukaemiasurvivalt(1;19)

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