Second-generation Sequencing Analysis of Ph / 中国实验血液学杂志

OBJECTIVE@#To screen the core genes of Philadelphia chromosome positive/Ph like T-cell acute lymphoblastic leukemia (Ph@*METHODS@#The WES/RNA-seq examination results of Ph@*RESULTS@#For Ph@*CONCLUSION@#There are obviously abnormal DNA damage repair pathways in children with Ph

Clinical features and prognosis of children with acute leukemias of ambiguous lineage under different diagnostic criteria / 中国当代儿科杂志

OBJECTIVES@#To study the clinical features and prognosis of children with acute leukemias of ambiguous lineage (ALAL) under different diagnostic criteria.@*METHODS@#A retrospective analysis was performed on the medical data of 39 children with ALAL who were diagnosed and treated from December 2015 to December 2019. Among the 39 children, 34 received treatment. According to the diagnostic criteria for ALAL by World Health Organization and European Group for the Immunological Characterization of Leukemias, the 39 children were divided into two groups: ALAL group (@*RESULTS@#The 34 children receiving treatment had a 3-year event-free survival (EFS) rate of 75%±9% and an overall survival rate of 88%±6%. The children treated with acute myeloid leukemia (AML) protocol had a 3-year EFS rate of 33%±27%, those treated with acute lymphoblastic leukemia (ALL) protocol had a 3-year EFS rate of 78%±10%, and those who had no remission after induction with AML protocol and then received ALL protocol had a 3-year EFS rate of 100%±0% (@*CONCLUSIONS@#ALL protocol has a better clinical effect than AML protocol in children with ALAL, and positive MRD after induction therapy suggests poor prognosis. Hyperleukocytosis and adverse genetic changes are not observed in children with myeloid expression, and such children tend to have a good prognosis, suggesting that we should be cautious to take it as ALAL in diagnosis and treatment.

Prognostic significance of measurable residual disease based on multiparameter flow cytometry in childhood acute myeloid leukemia / 中国当代儿科杂志

OBJECTIVES@#To study the prognostic value of measurable residual disease (MRD) for childhood acute myeloid leukemia (AML) by analyzing MRD-guided risk stratification therapy.@*METHODS@#A total of 93 children with AML were prospectively enrolled in this study. Chemotherapy with the 2015-AML-03 regimen was completed according to the risk stratification determined by genetic abnormality at initial diagnosis and MRD and bone marrow cytology after induction therapy I. Multiparameter flow cytometry was used to dynamically monitor MRD and analyze the prognostic effect of MRD on 3-year cumulative incidence of recurrence (CIR) rate, event-free survival (EFS) rate, and overall survival (OS) rate.@*RESULTS@#The 93 children with AML had a 3-year CIR rate of 48%±6%, a median time to recurrence of 11 months (range 2-32 months), a 3-year OS rate of 65%±6%, and a 3-year EFS rate of 50%±5%. After induction therapy I and intensive therapy I, the MRD-positive children had a significantly higher 3-year CIR rate and significantly lower 3-year EFS and OS rates than the MRD-negative children (@*CONCLUSIONS@#MRD has predictive value for the prognosis of children with AML. Based on the MRD-guided risk stratification therapy, reasonable application of chemotherapy may improve the overall prognosis of children with AML.

Serious adverse events associated with chemotherapy in children with acute lymphoblastic leukemia / 中国当代儿科杂志

OBJECTIVE@#To study the occurrence of serious adverse events (SAEs) related to chemotherapy with CCCG-ALL-2015 regimen in children with acute lymphoblastic leukemia (ALL) and the risk factors for death after the SAEs.@*METHODS@#A retrospective analysis was performed on the medical data of 734 children with ALL. They were treated with CCCG-ALL-2015 regimen from January 2015 to June 2019. The occurrence of SAEs during the treatment was investigated. The children with SAEs were divided into a death group with 25 children and a survival group with 31 children. A multivariate logistic regression analysis was used to analyze the risk factors for death after the SAEs.@*RESULTS@#Among the 734 children with ALL, 56 (7.6%) experienced SAEs (66 cases) after chemotherapy, among which 41 cases occurred in the stage of remission induction therapy. Of all 66 cases of SAEs, 46 (70%) were infection-related SAEs, including 25 cases of septic shock (38%), 20 cases of severe pneumonia (30%), and 1 case of severe chickenpox (2%), and 87% of the children with infection-related SAEs had neutrophil deficiency. The most common infection sites were blood and the lungs. The most common pathogens were Gram-negative bacteria, viruses, fungi, and Gram-positive bacteria. There were 16 cases (24%) of hemorrhage-related SAEs, with 11 cases of gastrointestinal bleeding (17%), 4 cases of pulmonary bleeding (6%), and 1 case of intracranial bleeding (2%). Of all 734 children with ALL, 66 (9.0%) died, among whom 25 died due to SAEs. The treatment-related mortality rate was 3.4%, and infection (72%) and bleeding (24%) were the main causes of death. Severe pneumonia was an independent risk factor for treatment-related death in ALL children (OR=4.087, 95%CI: 1.161-14.384, P=0.028).@*CONCLUSIONS@#SAEs often occur in the stage of remission induction therapy, and infection-related SAEs are more common in ALL children accepting chemotherapy with CCCG-ALL-2015 regimen. The development of severe pneumonia suggests an increased risk for death in these children.

Clinical significance of HA117, mdr1, mrp1, lrp and bcrp resistance genes expressions in children with acute leukemia / 中华实用儿科临床杂志

Objective To explore the expressions of the HA117,mdr1,mrp1,lrp and bcrp genes in bone marrow mononuclear cells(BMMNC) with acute leukemia (AL) and the clinical significance in AL.Methods Expressions of HA117,mdr1,mrp1,lrp and bcrp genes in 81 children with AL and 14 children with idiopathic thrombocytopenic purpura (ITP) were tested using semi-quantitative reverse transcriptase polymerase chain reaction (RT-PCR) technique.Results 1.The expressions of HA117,mdr1,mrp1,lrp and bcrp genes in AL patients were higher than that in ITP children.And the expressions of these genes in refractory-relapsed patients also higher than that in the initially diagnosed patients or remission patients.2.The first complete remission (CR1) rate in HA117 and lrp positive cases was lower than that in negative cases(all P ＜ 0.05),but the CR1 rates in mdr1,mrp1 and bcrp positive cases had no significant difference from it in negative cases(all P ＞ 0.05).3.Correlation analysis showed:there was no correlation between the expression of HA117 and mdr1 or HA117 and rnrp1 or HA117 and 1rp or HA117 and bcrp in childhood AL(r =0.031,0.319,0.203,0.176,11.178,all P ＞ 0.05).mdr1 and mrp1 or mdr1 and bcrp or lrp and bcrp had obviously positive correlation (r =0.260,0.308,0.317,all P ＜ 0.05).In refractory-relapsed group,HA117 and rndr1 or rnrp1 or lrp or bcrp had obviously perfect positive correlation.Conclusions The gene HA117 and mdr1,mrp1,lrp,bcrp may be associated with muhidrug resistance of AL in children,therefore the detection of HA117 expression is helpful in management of AL patients.Comparing to the mdr1 or mrp1 or bcrp genes,the genes of HA117 and lrp can better predict the multidrug resistance in childhood AL.