[Clinical Characteristics and Prognosis of Children with Hypodiploid B-cell Precursor Acute Lymphoblastic Leukemia].

Zhongguo Shi Yan Xue Ye Xue Za Zhi

Fujian Institute of Hematology, Fujian Provincial Key Laboratory, Fujian Medical University Union Hospital, Fuzhou 350001, Fujian Province, China.

Published: October 2024


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Article Abstract

Objective: To analyze the clinical characteristics and prognosis of children with hypodiploid B-cell precursor acute lymphoblastic leukemia (BCP-ALL).

Methods: The clinical data of 1 287 children with BCP-ALL admitted to five hospital in Fujian province from April 2011 to December 2020 were retrospectively analyzed. According to the results of chromosome karyotype, all the patients were grouped into hypodiploid subgroup and non-hypodiploid subgroup. The clinical characteristics, early treatment response [minimal residual disease (MRD) on middle stage of induction chemotherapy and end of induction chemotherapy] and long-term efficacy [overall survival (OS) and event-free survival (EFS)] were compared. The prognostic factors of hypodiploid BCP-ALL were further explored.

Results: Among 1 287 BCP-ALL patients, 28 patients (2.2%) were hypodiploid BCP-ALL. The proportion of patients with white blood cell count (WBC)≥50×10/L in the hypodiploid subgroup was significantly higher than that in the non-hypodiploid subgroup ( =0.004), while there was no statistically significant difference in gender ratio, age group at initial diagnosis, and early treatment response between the two groups (all >0.05). The 5-year EFS and OS rate of the hypodiploid subgroup were 75.0%(95% :66.8%-83.2%) and 77.8%(95% :69.8%-85.8%), respectively, which were lower than those of non-hypodiploid subgroup [EFS: 79.6%(95% :78.4%-80.8%); OS: 86.4%(95% :85.4%-87.5%)], but the difference was not statistically significant (all >0.05). Further subgroup analysis by risk stratification showed that the 5-year EFS and OS rates of the hypodiploid subgroup were significantly lower than those in the low-risk (LR) group [LR group EFS: 91.4% (95% :88.4%-93.6% ), < 0.001; OS: 94.7% (95% :92.1%-96.4%), < 0.001] ; it was similar to that of BCP-ALL children stratified into intermediate-risk (IR) excluding hypodiploid [IR group EFS: 79.4%(95% :74.9%-83.2%), =0.343; OS: 87.3%(95% :83.6%-90.2%), =0.111]; while was higher than that of EFS in HR group, but the difference was not statistically significant [HR group EFS: 58.7%(95% :52.6%-64.8%), =0.178. OS: 69.9%(95% :63.5%-75.4%), =0.417]. Univariate analysis showed that gender, age, white blood cell count, and MRD on middle stage of induction chemotherapy had no significant impact on OS and EFS; chromosome count< 40 was a risk factor for lower OS ( =0.026), but has no significant effect on EFS; MRD≥0.01% after induction therapy was a risk factor for lower OS and EFS ( =0.002, and 0.001, respectively).

Conclusion: Children with hypodiploid BCP-ALL have an intermediate prognosis, and MRD ≥0.01% after induction chemotherapy may be a risk factors for poor prognosis.

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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2024.05.008DOI Listing

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Article Synopsis
  • Hypodiploidy, characterized by a low modal number of chromosomes (45 or lower), is a known high-risk factor in pediatric acute lymphoblastic leukemia but hasn't been studied in pediatric acute myeloid leukemia (AML) until now.
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  • The results highlight that pediatric hypodiploid AML is a rare but challenging subgroup with poor prognosis, as even treatments like allogeneic
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