1.Clinical features and prognosis of acute B lymphoblastic leukemia children carrying a TCF3: : PBX1 fusion gene
Lulu HUANG ; Yunyan HE ; Yang LI ; Danna LIN ; Ning LIAO ; Yayun LING ; Lyuhong XU ; Xinyu LI ; Huirong MAI ; Ying WANG ; Wuqing WAN ; Ying LIU ; Yanlai TANG ; Xiaoli ZHANG ; Chuan TIAN ; Xiaofeng LI ; Qiwen CHEN ; Xingjiang LONG ; Liuhua LIAO ; Qiaoru LI ; Jianling CAI ; Zijun ZHEN ; Zhiguang LI ; Keyan YANG ; Qinlong ZHENG ; Lihua YANG
Chinese Journal of Applied Clinical Pediatrics 2025;40(7):497-502
Objective:To analyze the clinical features and prognosis of acute B lymphoblastic leukemia (B-ALL) children carrying a TCF3: : PBX1 fusion gene and to evaluate the prognostic value of this gene.Methods:Retrospective cohort study.A total of 2 164 B-ALL children aged 0-18 years diagnosed and treated at 19 pediatric centers from October 2016 to June 2022 were enrolled.They were divided into the positive group and the negative group according to whether they carried a TCF3: : PBX1 fusion gene.The clinical characteristics, treatment response, adverse reactions, and prognosis of the 2 groups of patients were analyzed.The rank sum and Kruskal-Wallis tests were used to compare two and more than two groups of numerical variables, respectively.Fisher′s exact test was used to compare categorical variables.Results:Among the 2 164 patients, 116 (5.4%) were TCF3: : PBX1 positive, of which 70 patients were female, accounting for 60.3%.There were 840 female patients in the TCF3: : PBX1-negative group, accounting for 41.0%.There was a significant difference in the ratio of females between the TCF3: : PBX1-positive and TCF3: : PBX1-negative groups ( P<0.001).No significant difference was observed in age of onset between the two groups( P>0.05).The proportion of bone marrow naive cells [54.00 (14.00, 76.50)% vs.29.00 (3.00, 68.00)%], white blood cell counts [25.30 (10.46, 60.94)×10 9/L vs.9.03 (4.38, 30.73)×10 9/L] and hemoglobin counts [82.00(63.00, 101.00) g/L vs.74.00(60.00, 90.00) g/L] in the TCF3: : PBX1-positive group were significantly higher than those in the negative group at the onset (all P<0.05).In terms of treatment response, the proportion of peripheral blood naive cells on Day 8 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group [2.00 (0, 9.00)% vs.0 (0, 2.00)%, P<0.001].The proportion of minimal residual disease <0.1% on Day 15 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group ( P=0.038).There were no significant differences in cumulative recurrence rate, treatment-related mortality (TRM), and overall survival (OS) between the TCF3: : PBX1-positive group and TCF3: : PBX1-negative group (all P>0.05).The cumulative recurrence risk of TCF3: : PBX1-positive patients was 9.646 times higher than that of ETV6: : RUNX1-positive patients with better prognosis( HR=9.646, 95% CI: 1.026-90.700, P=0.047).There were no significant differences in TRM and OS between TCF3: : PBX1-positive and ETV6: : RUNX1-positive patients (all P>0.05).A significant enrichment of PAX5 mutations was detected in TCF3: : PBX1-positive patients.Among the 7 high-risk TCF3: : PBX1-positive patients in a single center, 4 patients had PAX5 mutations, and this proportion was significantly higher than that in other patients ( P<0.001). Conclusions:B-ALL children carrying a TCF3: : PBX1 fusion gene have a high remission rate and good long-term prognosis after intensive chemotherapy.It is suggesting that TCF3: : PBX1-positive B-ALL patients should be rated at intermediate risk to receive intensive chemotherapy.
2.Clinical features and prognosis of acute B lymphoblastic leukemia children carrying a TCF3: : PBX1 fusion gene
Lulu HUANG ; Yunyan HE ; Yang LI ; Danna LIN ; Ning LIAO ; Yayun LING ; Lyuhong XU ; Xinyu LI ; Huirong MAI ; Ying WANG ; Wuqing WAN ; Ying LIU ; Yanlai TANG ; Xiaoli ZHANG ; Chuan TIAN ; Xiaofeng LI ; Qiwen CHEN ; Xingjiang LONG ; Liuhua LIAO ; Qiaoru LI ; Jianling CAI ; Zijun ZHEN ; Zhiguang LI ; Keyan YANG ; Qinlong ZHENG ; Lihua YANG
Chinese Journal of Applied Clinical Pediatrics 2025;40(7):497-502
Objective:To analyze the clinical features and prognosis of acute B lymphoblastic leukemia (B-ALL) children carrying a TCF3: : PBX1 fusion gene and to evaluate the prognostic value of this gene.Methods:Retrospective cohort study.A total of 2 164 B-ALL children aged 0-18 years diagnosed and treated at 19 pediatric centers from October 2016 to June 2022 were enrolled.They were divided into the positive group and the negative group according to whether they carried a TCF3: : PBX1 fusion gene.The clinical characteristics, treatment response, adverse reactions, and prognosis of the 2 groups of patients were analyzed.The rank sum and Kruskal-Wallis tests were used to compare two and more than two groups of numerical variables, respectively.Fisher′s exact test was used to compare categorical variables.Results:Among the 2 164 patients, 116 (5.4%) were TCF3: : PBX1 positive, of which 70 patients were female, accounting for 60.3%.There were 840 female patients in the TCF3: : PBX1-negative group, accounting for 41.0%.There was a significant difference in the ratio of females between the TCF3: : PBX1-positive and TCF3: : PBX1-negative groups ( P<0.001).No significant difference was observed in age of onset between the two groups( P>0.05).The proportion of bone marrow naive cells [54.00 (14.00, 76.50)% vs.29.00 (3.00, 68.00)%], white blood cell counts [25.30 (10.46, 60.94)×10 9/L vs.9.03 (4.38, 30.73)×10 9/L] and hemoglobin counts [82.00(63.00, 101.00) g/L vs.74.00(60.00, 90.00) g/L] in the TCF3: : PBX1-positive group were significantly higher than those in the negative group at the onset (all P<0.05).In terms of treatment response, the proportion of peripheral blood naive cells on Day 8 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group [2.00 (0, 9.00)% vs.0 (0, 2.00)%, P<0.001].The proportion of minimal residual disease <0.1% on Day 15 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group ( P=0.038).There were no significant differences in cumulative recurrence rate, treatment-related mortality (TRM), and overall survival (OS) between the TCF3: : PBX1-positive group and TCF3: : PBX1-negative group (all P>0.05).The cumulative recurrence risk of TCF3: : PBX1-positive patients was 9.646 times higher than that of ETV6: : RUNX1-positive patients with better prognosis( HR=9.646, 95% CI: 1.026-90.700, P=0.047).There were no significant differences in TRM and OS between TCF3: : PBX1-positive and ETV6: : RUNX1-positive patients (all P>0.05).A significant enrichment of PAX5 mutations was detected in TCF3: : PBX1-positive patients.Among the 7 high-risk TCF3: : PBX1-positive patients in a single center, 4 patients had PAX5 mutations, and this proportion was significantly higher than that in other patients ( P<0.001). Conclusions:B-ALL children carrying a TCF3: : PBX1 fusion gene have a high remission rate and good long-term prognosis after intensive chemotherapy.It is suggesting that TCF3: : PBX1-positive B-ALL patients should be rated at intermediate risk to receive intensive chemotherapy.
3.Results of the cancer screening program in urban areas in Shaanxi province of China, 2019-2020
Yong CHEN ; Benhua SONG ; Gang LI ; Peng CHEN ; Shanping HUANG ; Zijun LIAO ; Rui XU ; Yanrong LI
Chinese Journal of Oncology 2024;46(10):948-953
Objective:Analyze the cancer screening status of the cancer screening program in urban areas in Shaanxi province in 2019-2020.Methods:The early diagnosis and early treatment project for urban cancers carried out high-risk population screening for 5 types of high-incidence malignant tumors (breast cancer, lung cancer, upper gastrointestinal cancer, liver cancer, and colorectal cancer) in urban areas. Three prefecture-level cities in Shaanxi province with a population of over 1 million (Xi'an, Baoji, and Shangluo) were selected, and 4 communities with a relatively good working foundation were selected in each city. The general population aged 45-74 years was surveyed on the principles of informed consent and voluntariness, and high-risk groups identified through the questionnaire were further subjected to free endoscopy, ultrasound, CT, and other clinical screenings. The high-risk rates, screening compliance rates, and positive detection rates of the above 5 types of malignant tumors were analyzed.Results:A total of 19 632 people completed the survey effectively, with the proportion of male participants (40.0%) lower than that of females (60.0%). A total of 10 102 high-risk groups were identified, with an initial screening high-risk rate of 51.5%, and the high-risk rates for the 5 types of cancers were 24.1% for breast cancer, 28.6% for lung cancer, 9.1% for upper gastrointestinal cancer, 4.0% for liver cancer, and 20.0% for colorectal cancer. Among the 14 960 person-time initially assessed as high-risk, 5 129 person-time received clinical screening, with a screening compliance rate of 34.3%. The number of people receiving clinical screening and the screening compliance rates for the 5 types of cancers were 1 192 (41.9%) for breast cancer, 2 081 (37.1%) for lung cancer, 574 (32.0%) for upper gastrointestinal cancer, 404 (51.3%) for liver cancer, and 878 (22.3%) for colorectal cancer, with positive detection numbers and rates of 179 (15.0%) for breast, 289 (13.9%) for lung, 9 (1.6%) for upper gastrointestinal, 14 (3.5%) for suspected liver, and 67 (7.6%) for colorectal, respectively.Conclusion:The cancer screening status of the cancer screening program in urban areas in Shaanxi province is beneficial for the detection of precancerous lesions and early cancer patients, and improving the early diagnosis and treatment rate of patients, but the public participation rate is not high, and the project management model and technical plan need to be further improved.
4.Results of the cancer screening program in urban areas in Shaanxi province of China, 2019-2020
Yong CHEN ; Benhua SONG ; Gang LI ; Peng CHEN ; Shanping HUANG ; Zijun LIAO ; Rui XU ; Yanrong LI
Chinese Journal of Oncology 2024;46(10):948-953
Objective:Analyze the cancer screening status of the cancer screening program in urban areas in Shaanxi province in 2019-2020.Methods:The early diagnosis and early treatment project for urban cancers carried out high-risk population screening for 5 types of high-incidence malignant tumors (breast cancer, lung cancer, upper gastrointestinal cancer, liver cancer, and colorectal cancer) in urban areas. Three prefecture-level cities in Shaanxi province with a population of over 1 million (Xi'an, Baoji, and Shangluo) were selected, and 4 communities with a relatively good working foundation were selected in each city. The general population aged 45-74 years was surveyed on the principles of informed consent and voluntariness, and high-risk groups identified through the questionnaire were further subjected to free endoscopy, ultrasound, CT, and other clinical screenings. The high-risk rates, screening compliance rates, and positive detection rates of the above 5 types of malignant tumors were analyzed.Results:A total of 19 632 people completed the survey effectively, with the proportion of male participants (40.0%) lower than that of females (60.0%). A total of 10 102 high-risk groups were identified, with an initial screening high-risk rate of 51.5%, and the high-risk rates for the 5 types of cancers were 24.1% for breast cancer, 28.6% for lung cancer, 9.1% for upper gastrointestinal cancer, 4.0% for liver cancer, and 20.0% for colorectal cancer. Among the 14 960 person-time initially assessed as high-risk, 5 129 person-time received clinical screening, with a screening compliance rate of 34.3%. The number of people receiving clinical screening and the screening compliance rates for the 5 types of cancers were 1 192 (41.9%) for breast cancer, 2 081 (37.1%) for lung cancer, 574 (32.0%) for upper gastrointestinal cancer, 404 (51.3%) for liver cancer, and 878 (22.3%) for colorectal cancer, with positive detection numbers and rates of 179 (15.0%) for breast, 289 (13.9%) for lung, 9 (1.6%) for upper gastrointestinal, 14 (3.5%) for suspected liver, and 67 (7.6%) for colorectal, respectively.Conclusion:The cancer screening status of the cancer screening program in urban areas in Shaanxi province is beneficial for the detection of precancerous lesions and early cancer patients, and improving the early diagnosis and treatment rate of patients, but the public participation rate is not high, and the project management model and technical plan need to be further improved.
5.Stratified Treatment in Pediatric Anaplastic Large Cell Lymphoma: Result of a Prospective Open-Label Multiple-Institution Study
Tingting CHEN ; Chenggong ZENG ; Juan WANG ; Feifei SUN ; Junting HUANG ; Jia ZHU ; Suying LU ; Ning LIAO ; Xiaohong ZHANG ; Zaisheng CHEN ; Xiuli YUAN ; Zhen YANG ; Haixia GUO ; Liangchun YANG ; Chuan WEN ; Wenlin ZHANG ; Yang LI ; Xuequn LUO ; Zelin WU ; Lihua YANG ; Riyang LIU ; Mincui ZHENG ; Xiangling HE ; Xiaofei SUN ; Zijun ZHEN
Cancer Research and Treatment 2024;56(4):1252-1261
Purpose:
The risk stratification of pediatric anaplastic large cell lymphoma (ALCL) has not been standardized. In this study, new risk factors were included to establish a new risk stratification system for ALCL, and its feasibility in clinical practice was explored.
Materials and Methods:
On the basis of the non-Hodgkin’s lymphoma Berlin–Frankfurt–Munster 95 (NHL-BFM-95) protocol, patients with minimal disseminated disease (MDD), high-risk tumor site (multiple bone, skin, liver, and lung involvement), and small cell/lymphohistiocytic (SC/LH) pathological subtype were enrolled in risk stratification. Patients were treated with a modified NHL-BFM-95 protocol combined with an anaplastic lymphoma kinase inhibitor or vinblastine (VBL).
Results:
A total of 136 patients were enrolled in this study. The median age was 8.8 years. The 3-year event-free survival (EFS) and overall survival of the entire cohort were 77.7% (95% confidence interval [CI], 69.0% to 83.9%) and 92.3% (95% CI, 86.1% to 95.8%), respectively. The 3-year EFS rates of low-risk group (R1), intermediate-risk group (R2), and high-risk group (R3) patients were 100%, 89.5% (95% CI, 76.5% to 95.5%), and 67.9% (95% CI, 55.4% to 77.6%), respectively. The prognosis of patients with MDD (+), stage IV cancer, SC/LH lymphoma, and high-risk sites was poor, and the 3-year EFS rates were 45.3% (95% CI, 68.6% to 19.0%), 65.7% (95% CI, 47.6% to 78.9%), 55.7% (95% CI, 26.2% to 77.5%), and 70.7% (95% CI, 48.6% to 84.6%), respectively. At the end of follow-up, one of the five patients who received maintenance therapy with VBL relapsed, and seven patients receiving anaplastic lymphoma kinase inhibitor maintenance therapy did not experience relapse.
Conclusion
This study has confirmed the poor prognostic of MDD (+), high-risk site and SC/LH, but patients with SC/LH lymphoma and MDD (+) at diagnosis still need to receive better treatment (ClinicalTrials.gov number, NCT03971305).
6.Effects of total flavone of oldenlandia diffusa on the proliferation and apoptosis of hepatocellular carcinoma stem cell
Bowen YAO ; Yazhao LI ; Zijun LIAO ; Ye LU ; Xiang ZHANG ; Jiequn MA ; Qian LI ; Yanbing ZHANG
Journal of Xi'an Jiaotong University(Medical Sciences) 2023;44(3):389-395
【Objective】 To investigate the effects of total flavone of oldenlandia diffusa (FOD) on the proliferation and apoptosis of hepatocellular carcinoma (HCC) stem cells sorted from Huh7. 【Methods】 Human HCC cell lines Huh7 was cultured in vitro; CD133 positive (CD133+) stem cells in Huh7 cell line were sorted by flow cytometry, and stem cell markers such as Nanog, Oct4 and Sox2 were tested by Western blotting. CD133+-Huh7 was stimulated by different concentrations (0 μg/mL, 50 μg/mL, 100 μg/mL and 400 μg/mL) of FOD for different time (24 h, 48 h, 72 h and 96 h). CCK8 and plate cell cloning assay were used to detect the effect of FOD on CD133+-Huh7 proliferation while Annexin V-PE/7-AAD was used to detect the effect of FOD on CD133+-Huh7 apoptosis. Western blotting was used to detect the protein expressions of protein 53 (P53), factor associated suicide-Fas-associating protein with a novel death domain (Fas-FADD), B-cell lymphoma-2 (Bcl-2), Cleaved-Caspase3, and Bcl-2 associated X protein (Bax). 【Results】 More than 95% of stem cells were purified for further experiments. Cell proliferation of CD133+-Huh7 was significantly inhibited by FOD, with the significant suppression at the concentration of 100 μg/mL for 72 h compared with negative control group (P<0.05). The apoptosis rate was significantly upregulated than that in the negative control group (P<0.05). The protein expression of Bcl2 decreased while Bax and Cleaved-Caspae3 increased via FAS/FADDD and P53 axis. 【Conclusion】 FOD can significantly inhibit the proliferation and promote the apoptosis of CD133+-Huh7.
7.Recent advance in clinical drug therapy for Alzheimer's disease
Shang YI ; Henghui TAN ; Runtong LI ; Zijun LIAO ; Xin WEN ; Xiaoya GAO
Chinese Journal of Neuromedicine 2023;22(8):849-855
Alzheimer's disease (AD) is the first degenerative disease of the nervous system, but no drugs have been found to reverse AD progression. Starting from 2 major pathogenesis of AD, namely amyloid beta (Aβ) cascade and Tau protein, this study systematically reviews anti-Aβ or Tau protein AD new drugs that have entered clinical research; this study also expounds their clinical trial findings and mechanisms, and analyzes the reasons for their success or failure to provide a theoretical basis for AD drug exploitation.
8.Impact of hypophosphatemia on weaning from mechanical ventilation
Qiang BAO ; Minggen ZHOU ; Wenhua LIAO ; Lu PENG ; Zijun ZOU
Chinese Critical Care Medicine 2021;33(7):821-825
Objective:To explore the influence of hypophosphatemia on weaning from mechanical ventilation.Methods:An observational study was conducted. The medical records of 30 mechanical ventilated patients with hypophosphatemia admitted to intensive care unit of Sun Yat-sen Memorial Hospital of Sun Yat-sen University from January 2018 to August 2020 were analyzed; another 60 mechanical ventilated patients with normophosphatemia around the same time were enrolled as controls by 1∶2 case-control matching based on gender, age, acute physiology and chronic health evaluation Ⅱ (APACHEⅡ) score, sequential organ failure assessment (SOFA) score. And then the duration of invasive mechanical ventilation, times of spontaneous breathing trial (SBT), the diaphragmatic ultrasonography movement indexes, and outcome of weaning and prognosis during hospitalization were compared between the two groups. Receiver operator characteristic curve (ROC curve) was plotted to calculate the areas under ROC curve (AUC) and cut-off values of serum phosphorus for successful weaning and hospital survival. The correlations between the diaphragmatic ultrasonography movement indexes and serum phosphorus were analyzed by Pearson partial correlation analysis.Results:Compared with normophosphatemic group, the duration of invasive mechanical ventilation in hypophosphatemia group was significantly longer [days: 13.0 (7.0, 22.0) vs. 10.0 (5.5, 14.0), P < 0.05], and SBT attempts were more often [times: 3 (0, 5) vs. 1 (1, 2), P < 0.01], while the rate of successful weaning was lower (53.3% vs. 91.7%, P < 0.01), and the hospital mortality was higher (20.0% vs. 1.7%, P < 0.01). ROC curve analysis showed that serum phosphorus could predict successful weaning of mechanical ventilated patients, the AUC was 0.795, and the optimum cut-off value of serum phosphorus was 0.85 mmol/L with sensitivity of 73.2% and specificity of 84.2%. Serum phosphorus could predict hospital survival of mechanical ventilated patients, the AUC was 0.782, and the optimum cut-off value of serum phosphorus was 0.48 mmol/L with sensitivity of 81.9% and specificity of 85.7%. Compared with normophosphatemic group, diaphragm thickness at the end of inspiration (DTei), diaphragm thickness at the end of expiration (DTee), diaphragm thickening fraction (DTF), diaphragm excursion (DE) in hypophosphatemia group were all significantly decreased [DTei (cm): 0.19±0.07 vs. 0.27±0.08, DTee (cm): 0.14±0.05 vs. 0.19±0.06, DTF: (33.55±16.17)% vs. (45.04±18.66)%, DE (cm): 1.17±0.49 vs. 2.28±0.69, all P < 0.01]. Pearson partial correlation analysis showed that linear correlations were found between serum phosphorus and DTei, DTee, DTF, DE ( r values were 0.442, 0.351, 0.293, 0.628 respectively, all P < 0.01). Conclusions:Serum phosphorus may have correlation with the diaphragmatic ultrasonography movement indexes. Hypophosphatemia may impair the contractile properties of diaphragm, induce more SBT attempts and longer duration of invasive mechanical ventilation, and affect outcome of weaning and prognosis.
9. Expression and clinical significance of B7H3 in neuroblastoma tissues
Ru LIAO ; Xiaofei SUN ; Zijun ZHEN ; Juan WANG ; Dongsheng HUANG
Chinese Journal of Applied Clinical Pediatrics 2019;34(11):842-847
Objective:
To investigate the expression of B7H3 in neuroblastoma (NB) tissues and its relationship between clinical characteristics and prognosis of patients.
Methods:
The clinical data and pathological wax of 100 cases with neuroblastoma admitted from January 2000 to December 2015 in Sun Yet-Sen University Cancer Center were collected.The expression of B7H3 in tumor tissues was detected by immunohistochemistry (IHC) and then the expression of B7H3 and its relation to pathological parameters and survival rate of patients were analyzed.
Results:
(1) The positive rates of B7H3 in tumor tissues were 79% (79/100 cases), of which 37 were weak positive, 31 were mode-rate positive, 11 were strong positive, 58%(58/100 cases) showed low expression and 42%(42/100 cases) showed high expression.(2)The positive rate of B7H3 was positively correlated with the risk stratification, age, stage, primary site and
10.Construction of Nomogram prediction model for prognosis of gastric neuroendocrine neoplasms
ZUO Qianqian ; ZHANG Yanbing ; MA Jiequn ; LIAO Zijun ; LI Qian ; CHEN Yong
Chinese Journal of Cancer Biotherapy 2019;26(5):569-576
Objection: To analyze the factors affecting the prognosis of patients with gastric neuroendocrine neoplasms (G-NENs) by using the surveillance of National Cancer Institute (NCI) of America, Epidemiology and End Results (SEER) database, and to construct a prognostic Nomogram model for individualized prediction of prognosis in patients with G-NENs. Methods: The clinical data of 2720 G-NENs patients with complete follow-up data from 2010 to 2015 in the SEER database were collected. The prognostic Nomogram model was constructed based on independent risk factors determined by survival analysis. The consistency index (C-index) and calibration curve were used to evaluate its accuracy.Area under the curve (AUC) was used to compare the evaluation value between the Nomogram and the 7th edition of AJCC TNM staging. Results: The 1-, 3-, and 5-year survival rates of 2,720 patients with G-NENs were 88.14%, 79.09%, and 71.86%, respectively. Multivariate COX regression analysis showed that gender, age, marital status, other associated tumors, histological type, tumor grade, T stage, M stage, and surgery were independent risk factors affecting survival time of GNENs patients. The C-index of newly constructed Nomogram prediction model was 0.816, which was significantly higher than 0.702 of the 7thAJCC TNM staging (P<0.001), and the 1-, 3- and 5-year calibration curves showed a good agreement between predicted survival and actual survival. The AUC for 1-, 3- and 5-year survival by Nomogram prognostic model was 0.800, 0.811, and 0.820, which was higher than 0.650, 0.688 and 0.698 of the 7th AJCC TNM staging, and the differences were statistically significant (Z= 6.600, 8.085, 9.632, all P<0.0001). Conclusion: The Nomogram prediction model drawn in this study has a high prognostic value and can individually predict the survival rate of G-NENs patients, which is helpful for clinical treatment decision-making and clinical research options.

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