Prognostic Value and Outcome for ETV6/RUNX1-Positive Pediatric Acute Lymphoblastic Leukemia: A Report From the South China Children’s Leukemia Group

Qiu, Kunyin; Xu, Huilin; Luo, Xue-Qun; Mai, Huirong; Liao, Ning; Yang, Lihua; Zheng, Mincui; Wan, Wuqing; Wang, Xuedong; Liu, Riyang; Chen, Qi-wen; Chen, Huiqin; Sun, Xiaofei; Jiang, Hua; Long, Xiaoyan; Chen, Guohua; Li, Xinyu; Li, Changgang; Huang, Libin; Ling, Yayun; Lin, Danna; Wen, Chuan; Kuang, Wen-Yong; Feng, Xiaoqin; Ye, Zhong-lv; Wu, Bian; He, Xiaohong; Li, Qiao-ru; Wang, Lina; Kong, Xian-Ling; Xu, Lu-Hong; Li, Chi Kong; Fang, Jian-Pei

doi:10.3389/fonc.2021.797194

Cited by 11 publications

(18 citation statements)

References 39 publications

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“…2. Actually, the incidence of BCR-ABL1 positive ALL in this study was close to another report from the South China Children’s Leukemia Group ( 27 ), suggesting that the positive rate of BCR-ABL1 in the population of South China may just be higher than that in the regions involved in other protocols. In addition, the incidence of induction failure (IF) was higher than that reported in other protocols ( 28 ).…”

Section: Discussionsupporting

confidence: 85%

“…Moreover, research in recent years have shown that patients with central nervous system leukemia (CNSL), T-cell immunophenotype, low hypodiploid, BCR-ABL1 positive, E2A-PBX1/TCF3-PBX1 positive, or MLL rearrangements also had a poor prognosis ( 14 – 17 ). Conversely, patients with ETV6-RUNX1/TEL-AML1 positive had a better prognosis ( 27 , 40 ). In the present study, we found that T-cell immunophenotype was a risk factor for EFS, while ETV6-RUNX1/TEL-AML1 was a protective factor, which were agreement with previous reports.…”

Section: Discussionmentioning

confidence: 99%

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A Nomogram for Predicting Event-Free Survival in Childhood Acute Lymphoblastic Leukemia: A Multicenter Retrospective Study

Zhou

Yang

et al. 2022

Front. Oncol.

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ObjectiveEven though childhood acute lymphoblastic leukemia (ALL) has an encouraging survival rate in recent years, some patients are still at risk of relapse or even death. Therefore, we aimed to construct a nomogram to predict event-free survival (EFS) in patients with ALL.MethodChildren with newly diagnosed ALL between October 2016 and July 2021 from 18 hospitals participating in the South China children’s leukemia Group (SCCLG) were recruited and randomly classified into two subsets in a 7:3 ratio (training set, n=1187; validation set, n=506). Least absolute shrinkage and selection operator (LASSO) and multivariate Cox regression analysis were adopted to screen independent prognostic factors. Then, a nomogram can be build based on these prognostic factors to predict 1-, 2-, and 3-year EFS. Concordance index (C-index), area under the curve (AUC), calibration curve, and decision curve analysis (DCA) were used to evaluate the performance and clinical utility of nomogram.ResultThe parameters that predicted EFS were age at diagnosis, white blood cell at diagnosis, immunophenotype, ETV6-RUNX1/TEL-AML1 gene fusion, bone marrow remission at day 15, and minimal residual disease at day 15. The nomogram incorporated the six factors and provided C-index values of 0.811 [95% confidence interval (CI) = 0.792-0.830] and 0.797 (95% CI = 0.769-0.825) in the training and validation set, respectively. The calibration curve and AUC revealed that the nomogram had good ability to predict 1-, 2-, and 3-year EFS. DCA also indicated that our nomogram had good clinical utility. Kaplan–Meier analysis showed that EFS in the different risk groups stratified by the nomogram scores was significant differentiated.ConclusionThe nomogram for predicting EFS of children with ALL has good performance and clinical utility. The model could help clinical decision-making.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 99%

A Nomogram for Predicting Event-Free Survival in Childhood Acute Lymphoblastic Leukemia: A Multicenter Retrospective Study

Zhou

Yang

et al. 2022

Front. Oncol.

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“…The South China Children’s Leukemia Group (SCCLG)-ALL-2016 is a prospective, multi-institutional clinical trial involving 18 major hospitals/medical centers ( 9 , 10 ). We designed the SCCLG-ALL-2016 collaborative protocol based on the backbones of Berlin-Frankfurt-Münster (BFM) ALL-IC-2009 and Guangdong (GD)-2008-ALL ( 11 , 12 ).…”

Section: Introductionmentioning

confidence: 99%

Prognostic significance of CNSL at diagnosis of childhood B-cell acute lymphoblastic leukemia: A report from the South China Children’s Leukemia Group

Liao

et al. 2022

Front. Oncol.

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ObjectivesThe prognostic significance of acute lymphoblastic leukemia (ALL) patients with central nervous system leukemia (CNSL) at diagnosis is controversial. We aimed to determine the impact of CNSL at diagnosis on the clinical outcomes of childhood B-cell ALL in the South China Children’s Leukemia Group (SCCLG).MethodsA total of 1,872 childhood patients were recruited for the study between October 2016 and July 2021. The diagnosis of CNSL depends on primary cytological examination of cerebrospinal fluid, clinical manifestations, and imaging manifestations. Patients with CNSL at diagnosis received two additional courses of intrathecal triple injections during induction.ResultsThe frequency of CNLS at the diagnosis of B-cell ALL was 3.6%. Patients with CNSL at diagnosis had a significantly higher mean presenting leukocyte count (P = 0.002) and poorer treatment response (P <0.05) compared with non-CNSL patients. Moreover, CNSL status was associated with worse 3-year event-free survival (P = 0.030) and a higher risk of 3-year cumulative incidence of relapse (P = 0.008), while no impact was observed on 3-year overall survival (P = 0.837). Multivariate analysis revealed that CNSL status at diagnosis was an independent predictor with a higher cumulative incidence of relapse (hazard ratio = 2.809, P = 0.016).ConclusionCNSL status remains an adverse prognostic factor in childhood B-cell ALL, indicating that additional augmentation of CNS-directed therapy is warranted for patients with CNSL at diagnosis.

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“…Various chromosomal alterations in ALL are important in driving chromosomal gains and losses that may be associated with the target therapy, survival and relapse of the disease ( 5 , 11 , 12 ). Chromosomal abnormalities such as t (4;11) (q21;q23) are associated with poor prognosis ( 13 , 14 ), whereas patients with t(12;21)(p13;q22) translocation have a better survival rate ( 15 , 16 ). A subtype of ALL carrying t(9;22)(q34;q11.2) translocation had a poor prognosis, which has been improved significantly by the combination of tyrosine kinase and chemotherapy strategies ( 17 – 19 ).…”

Section: Introductionmentioning

confidence: 99%

An easy-to-use nomogram predicting overall survival of adult acute lymphoblastic leukemia

et al. 2022

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Adult acute lymphoblastic leukemia (ALL) is heterogeneous both biologically and clinically. The outcomes of ALL have been improved with the application of children-like regimens and novel agents including immune therapy in young adults. The refractory to therapy and relapse of ALL have occurred in most adult cases. Factors affecting the prognosis of ALL include age and white blood cell (WBC) count at diagnosis. The clinical implications of genetic biomarkers, including chromosome translocation and gene mutation, have been explored in ALL. The interactions of these factors on the prediction of prognosis have not been evaluated in adult ALL. A prognostic model based on clinical and genetic abnormalities is necessary for clinical practice in the management of adult ALL. The newly diagnosed adult ALL patients were divided into the training and the validation cohort at 7:3 ratio. Factors associated with overall survival (OS) were assessed by univariate/multivariate Cox regression analyses and a signature score was assigned to each independent factor. A nomogram based on the signature score was developed and validated. The receiver operating characteristic (ROC) curve, calibration curve, and decision curve analysis (DCA) were used to assess the performance of the nomogram model. This study included a total of 229 newly diagnosed ALL patients. Five independent variables including age, WBC, bone marrow (BM) blasts, MLL rearrangement, and ICT gene mutations (carried any positive mutation of IKZF1, CREBBP and TP53) were identified as independent adverse factors for OS evaluated by the univariate, Kaplan-Meier survival and multivariate Cox regression analyses. A prognostic nomogram was built based on these factors. The areas under the ROC curve and calibration curve showed good accuracy between the predicted and observed values. The DCA curve showed that the performance of our model was superior to current risk factors. A nomogram was developed and validated based on the clinical and laboratory factors in newly diagnosed ALL patients. This model is effective to predict the overall survival of adult ALL. It is a simple and easy-to-use model that could efficiently predict the prognosis of adult ALL and is useful for decision making of treatment.

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Prognostic Value and Outcome for ETV6/RUNX1-Positive Pediatric Acute Lymphoblastic Leukemia: A Report From the South China Children’s Leukemia Group

Cited by 11 publications

References 39 publications

A Nomogram for Predicting Event-Free Survival in Childhood Acute Lymphoblastic Leukemia: A Multicenter Retrospective Study

A Nomogram for Predicting Event-Free Survival in Childhood Acute Lymphoblastic Leukemia: A Multicenter Retrospective Study

Prognostic significance of CNSL at diagnosis of childhood B-cell acute lymphoblastic leukemia: A report from the South China Children’s Leukemia Group

An easy-to-use nomogram predicting overall survival of adult acute lymphoblastic leukemia

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