Clinical features and prognostic impact of TCF3–PBX1 in childhood acute lymphoblastic leukemia: A single-center retrospective study of 837 patients from China

Jia, Ming; Hu, Bo-Fei; Xu, Xiao‐Jun; Zhang, Jingying; Li, Sisi; Tang, Yongmin

doi:10.1016/j.currproblcancer.2021.100758

Cited by 18 publications

(18 citation statements)

References 28 publications

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“…Similarly, male sex, CNS leukemia, and MRD ≥0.01% at day 19 and day 46 were associated with inferior event‐free survival among our patients with TCF3::PBX1 fusion. Male sex was also identified to be an independent adverse prognostic factor in patients with this genotype in another Chinese study 17 . The finding of testicular leukemia at diagnosis as an independent adverse prognostic factor in patients with TCF3::PBX1 ALL is novel and was not previously recognized probably because of the lack of large studies.…”

Section: Discussionmentioning

confidence: 90%

Prognostic factors of childhood acute lymphoblastic leukemia with TCF3::PBX1 in CCCG‐ALL‐2015: A multicenter study

Zhang

Wan

Wang

et al. 2023

Cancer

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Background Contemporary risk‐directed treatment has improved the outcome of patients with acute lymphoblastic leukemia (ALL) and TCF3::PBX1 fusion. In this study, the authors seek to identify prognostic factors that can be used to further improve outcome. Methods The authors studied 384 patients with this genotype treated on Chinese Children's Cancer Group ALL‐2015 protocol between January 1, 2015 and December 31, 2019. All patients provisionally received intensified chemotherapy in the intermediate‐risk arm without prophylactic cranial irradiation; those with high minimal residual disease (MRD) ≥1% at day 46 (end) of remission induction were candidates for hematopoietic cell transplantation. Results The overall 5‐year event‐free survival was 84.4% (95% confidence interval [CI], 80.6–88.3) and 5‐year overall survival 88.9% (95% CI, 85.5–92.4). Independent factors associated with lower 5‐year event‐free survival were male sex (80.4%, [95% CI, 74.8–86.4] vs. 88.9%, [95% CI, 84.1–93.9] in female, p = .03) and positive day 46 MRD (≥0.01%) (62.1%, [95% CI, 44.2–87.4] vs. 87.1%, [95% CI, 83.4–90.9] in patients with negative MRD, p < .001). The presence of testicular leukemia at diagnosis (n = 10) was associated with particularly dismal 5‐year event‐free survival (33.3% [95% CI, 11.6–96.1] vs. 83.0% [95% CI, 77.5–88.9] in the other 192 male patients, p < .001) and was an independent risk factor (hazard ratio [HR], 5.7; [95% CI, 2.2–14.5], p < .001). Conclusions These data suggest that the presence of positive MRD after intensive remission induction and testicular leukemia at diagnosis are indicators for new molecular therapeutics or immunotherapy in patients with TCF3::PBX1 ALL.

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Section: Discussionmentioning

confidence: 90%

Prognostic factors of childhood acute lymphoblastic leukemia with TCF3::PBX1 in CCCG‐ALL‐2015: A multicenter study

Zhang

Wan

Wang

et al. 2023

Cancer

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“…Similarly, male sex and MRD ≥ 0.01% at Day 19 and Day 46 were associated with inferior event-free survival among our patients with TCF3-PBX1 fusion. Male sex was also identified to be an independent adverse prognostic factor in patients with this genotype in another Chinese study [17]. The finding of testicular leukemia at diagnosis as an independent adverse prognostic factor in patients with TCF3-PBX1 ALL is novel and was not previously recognized probably because of the lack of large studies.…”

Section: Discussionmentioning

confidence: 91%

“…This genotype had historically been associated with a poor prognosis [9][10][11] and increased central nervous system (CNS) relapse [12]. Contemporary risk-directed treatments have improved its prognosis [13][14][15][16][17][18], to the extent that many study groups exclude this genotype in their risk stratification. With the availability of new targeted therapies, we study a large cohort of children with this genotype who were treated with risk-directed therapy in a recently completed Chinese Children's Cancer Group ALL-2015 protocol (CCCG-ALL-2015) [19,20], to seek prognostic factors that could be useful to further improve outcome.…”

Section: Introductionmentioning

confidence: 99%

Prognostic factors of childhood acute lymphoblastic leukemia with TCF3-PBX1 in CCCG-ALL-2015: a multi-center study

Zhang

Wan

Wang

et al. 2022

Preprint

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To identify prognostic factors that can be used to further improve outcome, we studied 384 patients with this genotype treated on Chinese Children’s Cancer Group acute lymphoblastic leukemia (ALL) -2015 protocol between 1 January 2015 and 31 December 2019. All patients provisionally received intensified chemotherapy in the intermediate-risk. The overall 5-year event-free survival (EFS) was 84.4% (95% confidence interval [CI], 80.6-88.3). Independent factors associated with lower 5-year EFS were male sex (80.4%, [95% CI, 74.8-86.4] vs. 88.9%, [95% CI, 84.1-93.9] in female, p = 0.033) and positive Day 46 minimal residual disease (MRD) (≥ 0.01%) (62.1%, [95% CI, 44.2-87.4] vs. 87.1%, [95% CI, 83.4-90.9] in patients with negative MRD, p < 0.001). The presence of testicular leukemia at diagnosis (n=10) was associated with particularly poorer 5-year EFS (33.3% [95% CI, 11.6-96.1] vs. 83.0% [95% CI, 77.5-88.9] in the other 192 male patients, P < 0.001) and was an independent risk factor (HR, 5.7, [95% CI, 2.2-14.5], p < 0.001). These data suggest that the presence of positive MRD after intensive remission induction and testicular leukemia at diagnosis are indications for new molecular therapeutics or immunotherapy in patients with TCF3-PBX1 ALL.

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“…Most TCF3/PBX1 -positive patients have been shown to have adverse prognostic factors, such as high WBC count and older age ( 25 , 26 ). In the NPCLC-ALL-2008 trial, the complete response, prednisone response, and recurrence rates were not significantly different between TCF3/PBX1 -positive and TCF3/PBX1 -negative patients ( 27 ). However, the high proportion of TCF3/PBX1 -positive patients in the DPR group in this study was not consistent with the single-center results of the NPCLC-ALL-2008 trial, which may be related to the difference in the sample size.…”

Section: Discussionmentioning

confidence: 96%

Prognostic significance of steroid response in pediatric acute lymphoblastic leukemia: The CCCG-ALL-2015 study

Chu

Cai

et al. 2022

Front. Oncol.

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IntroductionWhether steroid response is an independent risk factor for acute lymphoblastic leukemia (ALL) is controversial. This study aimed to investigate the relationship between response to dexamethasone and prognosis in children with ALL.MethodsWe analyzed the data of 5,161 children with ALL who received treatment in accordance with the Chinese Children’s Cancer Group ALL-2015 protocol between January 1, 2015, and December 31, 2018, in China. All patients received dexamethasone for 4 days as upfront window therapy. Based on the peripheral lymphoblast count on day 5, these patients were classified into the dexamethasone good response (DGR) and dexamethasone poor response (DPR) groups. A peripheral lymphoblast count ≥1× 109/L indicated poor response to dexamethasone.ResultsThe age, white blood cell counts, prevalence of the BCR/ABL1 and TCF3/PBX1 fusion genes, and rates of recurrence in the central nervous system were higher in the DPR than in the DGR group (P<0.001). Compared to the DPR group, the DGR group had a lower recurrence rate (18.6% vs. 11%) and higher 6-year event-free survival (73% vs. 83%) and overall survival (86% vs. 92%) rates; nevertheless, subgroup analysis only showed significant difference in the intermediate-risk group (P<0.001).DiscussionResponse to dexamethasone was associated with an early treatment response in our study. In the intermediate-risk group, dexamethasone response added a prognostic value in addition to minimal residual disease, which may direct early intervention to reduce the relapse rate.

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Clinical features and prognostic impact of TCF3–PBX1 in childhood acute lymphoblastic leukemia: A single-center retrospective study of 837 patients from China

Cited by 18 publications

References 28 publications

Prognostic factors of childhood acute lymphoblastic leukemia with TCF3::PBX1 in CCCG‐ALL‐2015: A multicenter study

Prognostic factors of childhood acute lymphoblastic leukemia with TCF3::PBX1 in CCCG‐ALL‐2015: A multicenter study

Prognostic factors of childhood acute lymphoblastic leukemia with TCF3-PBX1 in CCCG-ALL-2015: a multi-center study

Prognostic significance of steroid response in pediatric acute lymphoblastic leukemia: The CCCG-ALL-2015 study

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