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[Clinical characteristics and prognostic analysis of pediatric pro-B cell acute lymphoblastic leukemia]
Yu-Juan Xue1, Ai-Dong Lu1, Yu Wang1
1Department of Pediatrics, People's Hospital, Peking University, Beijing 100044, China.
Insights
Pediatric pro-B acute lymphoblastic leukemia (pro-B-ALL) shows diverse clinical and biological traits. Minimal residual disease (MRD) at 3 months is a key predictor of long-term outcomes in pro-B-ALL patients.
Area of Science:
- Pediatric Oncology
- Hematology
- Molecular Biology
Background:
- Pro-B cell acute lymphoblastic leukemia (pro-B-ALL) is a distinct subtype of pediatric ALL.
- Understanding its clinical and biological characteristics is crucial for improving patient outcomes.
Purpose of the Study:
- To investigate the clinical-biological features of pediatric pro-B-ALL.
- To identify prognostic factors influencing the survival of these patients.
Main Methods:
- Retrospective analysis of 64 pediatric patients diagnosed with pro-B-ALL.
- Evaluation of clinical data, immunological markers, genetic alterations (including MLL rearrangements), and minimal residual disease (MRD).
Main Results:
- Pro-B-ALL accounted for 6.23% of pediatric ALL cases.
- MLL rearrangements were common (34%), associated with specific immunophenotypic profiles.
- Five-year overall survival (OS) and event-free survival (EFS) were 85% and 78%, respectively.
- MRD levels ≥ 0.1% at 3 months post-chemotherapy independently predicted poorer OS and EFS.
Conclusions:
- Pediatric pro-B-ALL exhibits significant clinical and biological heterogeneity.
- Immunophenotypic markers, genetic alterations, and early MRD response are vital for predicting long-term prognosis in pro-B-ALL.
Objective:
To explore the clinical-biological characteristics and prognosis of pediatric pro-B cell acute lymphoblastic leukemia (pro-B-ALL).
Methods:
A total of 64 patients aged less than 18 years old with pro-BALL were enrolled. Clinical characteristics, therapeutic effect and prognostic factors were retrospectively analyzed.
Results:
Pro-B-ALL occurred in 6.23% (64/1 028) of pediatric ALL. Among the 64 patients, 35 were male and 29 were female. The median age was 7.0 years (range 0.4-16.0 years) at diagnosis, of which 39% and 6% were ≥ 10 years old and < 1 year old respectively. The median WBC count was 25.5×109/L[range (0.4-831.9)×109/L], of which 35.9% were ≥ 50×109/L. MLL-r positivity was the most frequent genetic alteration in pro-B ALL, occurring in 34% of patients, with lower frequency of CD22 and CD13 expression and higher frequency of CD7 expression, while lower frequency of CD33 expression was found in patients with MLL-AF4 positivity. At a median follow-up of 60.0 months (range 4.9-165.3 months), the estimated 5-year overall survival (OS) and event-free survival (EFS) in the 64 patients were (85±5)% and (78±5)% respectively. Cox proportional hazards regression analysis identified MRD ≥ 0.1% at 3 months after chemotherapy as an independent adverse prognostic factor for both 5-year OS and EFS.
Conclusions:
Pediatric pro-B ALL is a heterogeneous disease with clinical and biological diversity. Biological characteristics, such as immunological markers, genetic alterations, and MRD at 3 months after chemotherapy may be important factors for the long-term prognosis.
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