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1.
Article in Chinese | WPRIM | ID: wpr-880028

ABSTRACT

OBJECTIVE@#To analyze the outcomes of the children suffered from philadelphia chromosome positive acute lymphoblastic leukemia (Ph@*METHODS@#21 cases of firstly diagnosed Ph@*RESULTS@#Among 21 patients, 17 were male and 4 were female with a median age of 8 years old (range, 4-12 years), the median follow-up time was 30 moths (range, 10-133 months). All the patients were treated with chemotherapy induced by the high-risk project of CCLG-ALL 2008. Among 14 patients treated with TKI plus chemotherapy, nine patients achieved complete remission. During 3 months after treatment, patients without complete molecular response or with the second complete remission and intensity desire of transplantation were treated with allo-HSCT, among 9 patients with allo-HSCT, six patients achieved long term survival.@*CONCLUSION@#At TKI era, TKI combined with strong chemotherapy can make Ph


Subject(s)
Aged , Child , Female , Hematopoietic Stem Cell Transplantation , Humans , Infant , Male , Philadelphia Chromosome , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Protein Kinase Inhibitors , Retrospective Studies
2.
Article in Chinese | WPRIM | ID: wpr-846457

ABSTRACT

Objective: To establish a method for determining the contents of schisandrin, schisandrol B, cryptotanshinone, tanshinone I and tanshinone IIA, γ-schisandrin in Zaoren Anshen Capsules (ZAC), and provide scientific method for quality control of ZAC. Methods: HPLC was used by using Thermo 120Å-C18 column, with the mobile phase consisted of acetonitrile and 0.1% acetic acid solution with gradient elution for simultaneous determination of six main index components. The detection wavelengths were set at 250 nm for schisandrin, schisandrol B, γ-schisandrin and 270 nm for cryptotanshinone, tanshinone I, and tanshinone IIA, flow rate was 1.0 mL/min, and column temperature was 30℃. Results: Schisandrin, schisandrol B, γ-schisandrin, cryptotanshinone, tanshinone I, and tanshinone IIA showed good the linear ranges relationships in the range of 4.7-3 153.6 ng (r = 0.999 9), 7.864-314.500 ng (r = 0.999 9), 14.4-1 256.9 ng (r = 0.999 9), 15.1-1 103.8 ng (r = 0.999 9), 15.3-1 532.0 ng (r = 0.999 9) and 6.134-204.500 ng (r = 0.999 9), respectively. The average recoveries were 100.4%, 98.7%, 99.4%, 100.0%, 99.3% and 100.2%, RSD were 1.4%, 2.7%, 2.2%, 2.2%, 2.5% and 2.1%, respectively. The contents of each index component in 8 batches of sample were schisandrin 1.545 7-1.909 9 mg/g, schisandrol B 0.129 8-0.235 1 mg/g, cryptotanshinone 0.508 4-0.523 4 mg/g, tanshinone I 0.111 7-0.122 3 mg/g, tanshinone IIA 0.755 8-0.874 4 mg/g, γ-schisandrin 0.120 2-0.190 1 mg/g. Conclusion: The established analytical method is highly sensitive with strong specificity and it can be used efficiently in the quality control of ZAC.

3.
Article in Chinese | WPRIM | ID: wpr-879771

ABSTRACT

OBJECTIVE@#To study the pharmacokinetic characteristics, clinical effect, and safety of pegylated recombinant human granulocyte colony-stimulating factor (PEG-rhG-CSF) in children with acute lymphoblastic leukemia (ALL).@*METHODS@#A prospective study was performed on children with ALL who cyclophosphamide, cytarabine, and 6-mercaptopurine were used for consolidation therapy. PEG-rhG-CSF (PEG-rhG-CSF group) or rhG-CSF (rhG-CSF group) was injected after chemotherapy. The plasma concentration of PEG-rhG-CSF was measured, and clinical outcome and safety were observed for both groups.@*RESULTS@#A total of 17 children with ALL were enrolled, with 9 children in the PEG-rhG-CSF group and 8 children in the rhG-CSF group. In the PEG-rhG-CSF group, the peak concentration of PEG-rhG-CSF was 348.2 ng/mL (range 114.7-552.0 ng/mL), the time to peak was 48 hours (range 12-72 hours), and the half life was 14.1 hours (range 11.1-18.1 hours). The plasma concentration curve of PEG-rhG-CSF was consistent with the mechanism of neutrophil-mediated clearance. Compared with the rhG-CSF group, the PEG-rhG-CSF group had a significantly shorter median time to absolute neutrophil count (ANC) recovery (P0.05).@*CONCLUSIONS@#The pharmacokinetic characteristics of PEG-rhG-CSF in children with ALL receiving consolidation chemotherapy are consistent with the mechanism of neutrophil-mediated clearance, with a short half life and fast recovery of ANC, and there are no significant differences in safety between PEG-rhG-CSF and rhG-CSF.


Subject(s)
Child , Granulocyte Colony-Stimulating Factor/therapeutic use , Humans , Neutropenia , Polyethylene Glycols , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Prospective Studies , Recombinant Proteins
4.
Journal of Experimental Hematology ; (6): 1831-1836, 2020.
Article in Chinese | WPRIM | ID: wpr-879979

ABSTRACT

OBJECTIVE@#To investigate the consistency between FCM and PCR on the detecting of MRD in TCF3-PBX1@*METHODS@#55 cases of paediatric TCF3-PBX1@*RESULTS@#Among the 55 children with TCF3-PBX1@*CONCLUSION@#The detection result of MRD in TCF3-PBX1 detect by FCM and PCR shows better consistency. MRD positivity detected by FCM at the end of induction therapy (day 33) predicts a high risk of relapse in TCF3-PBX1 ALL patients.


Subject(s)
Adolescent , Bone Marrow , Child , Child, Preschool , Female , Humans , Male , Neoplasm, Residual , Oncogene Proteins, Fusion/genetics , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Prognosis , Recurrence
5.
Article in Chinese | WPRIM | ID: wpr-781460

ABSTRACT

OBJECTIVE@#To analyze the clinical efficacy and side effects of reduced-dose of cyclophosphamide combined cyclosporine A for severe aplastic anemia(SAA) children.@*METHODS@#Ten pediatric patients with SAA from January 2008 to May 2012 were enrolled. All the patients were treated with reduced dose of cyclophosphamide combined cyclosporine A. The dose of cyclophosphamide was 30 mg/(kg·d)×4 d, the dose of cyclosporine A gradually increased >15 mg/L accroding to the blood concentration.@*RESULTS@#The median follow-up time of the 10 pediatric patients was 100 months (6-126 months). Among 10 children with SAA, 4 cases achieved complete response(CR), 3 cases obtained partial response (PR) and the overall response rate was 70%, the remaining 3 cases showed no response (NR). One refractory patient treated by cyclophosphamide was progressed to paroxysmal nocturnal hemoglobinuria(PNH) at 25 months and was dead at 42 months after therapy.@*CONCLUSION@#The results show that reduced-dose cyclophosphamide (30 mg/kg·d for 4 consecutive days) combinated with CsA (initial dose 4 mg/kg·d, and drugvallery concentration >150 ng/ml) can make 7 of 10 children with severe aplastic anemia achieve complete response or partial response, and this regimen may be the second line regimen selected for some SAA children.

6.
Journal of Experimental Hematology ; (6): 1075-1080, 2020.
Article in Chinese | WPRIM | ID: wpr-827158

ABSTRACT

OBJECTIVE@#To study the long-term efficacy of CCLG-ALL2008 protocol used for treatment of childhood acute lymphoblastic leukemia (ALL).@*METHODS@#Nine hundred and forty children with newly diagnosed ALL from January 2008 to April 2015 were treated with CCLG-ALL2008 protocol. Overall survival (OS) and event-free survival (EFS) rates were estimated by the Kaplan-Meier method. Cox proportional hazards model was used for analyses of prognostic factors.@*RESULTS@#Among the 940 newly diagnosed ALL patients, 570 patients were male, and 370 patients were female, the median age of onset was 5 years old (from 1 to 15 years old). The complete reaction rate (CR) was 96.7%. Survival analysis of 916 ALL patients with CR estimated by follow up [ (median follow up period 64 months (from 3 to 123 months) ] showed that, the expected 10 year OS rate was (78.6±1.5)% and the EFS rate was (66.0±1.8)%. The long-term OS rate of standard risk, intermediate risk and high risk patients was (93.0±1.5)%, (77.6±2.7)%, and (59.3±3.7)%, respectively, and the long-term EFS rate in standard risk, intermediate risk and high risk patients was (84.2±2.2)%. (67.8±2.9)%, and (42.1±3.9)% respectively. 10 year OS rate in B-ALL patients (79.8±1.6)% was significantly higher than that in T-ALL patients (53.5±6.3)% (P<0.01). Among of all the patients, patients 201 (21.9%) relapsed, the median relapse time was 19 months (from 2 months to 81 months). The 10 year EFS rate was (81.7±3.7)% in the patients with MRD rate <0.01% after induction therapy, which was significantly higher than that in the patients with MRD rate>0.01% (48.4±9.8)%.@*CONCLUSION@#The therapeutic efficacy of the CCLG-ALL2008 protocol is closed to the level of supior study group in the world. Risk stratification can improve the outcome for childhood ALL. Immunophenotyping shows the outcome of B-ALL is better than that of T-ALL. MRD negative patients after induction therapy shows better prognosis compared with that of MRD positive patients.


Subject(s)
Adolescent , Antineoplastic Combined Chemotherapy Protocols , Child , Child, Preschool , China , Disease-Free Survival , Female , Humans , Infant , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Drug Therapy , Prognosis , Prospective Studies , Treatment Outcome
7.
Article in Chinese | WPRIM | ID: wpr-775109

ABSTRACT

OBJECTIVE@#To study the association of platelet level at diagnosis with prognosis in children with acute lymphoblastic leukemia (ALL).@*METHODS@#A total of 892 children with ALL who underwent chemotherapy with the CCLG-ALL 2008 regimen were enrolled. According to the platelet count at diagnosis, these children were divided into normal platelet count group (platelet count ≥100×109/L; n=263) and thrombocytopenia group (platelet count 0.05). The normal platelet count group still had a significantly higher 10-year EFS rate than the thrombocytopenia group after the children with MLL gene rearrangement were excluded (P0.05). The <20×10/L subgroup had significantly lower 10-year EFS and OS rates than the normal platelet count group, the (50- <100)×10/L subgroup, and the (20- <50)×10/L subgroup (P<0.05). After the children with MLL gene rearrangement were excluded, the <20×10/L subgroup still had significantly lower 10-year EFS and OS rates than the normal platelet count group, the (50-<100)×10/L subgroup, and the (20- <50)×10/L subgroup (P<0.05).@*CONCLUSIONS@#ALL children with MLL gene rearrangement often have the clinical manifestation of thrombocytopenia. Platelet level at diagnosis is associated with the prognosis of ALL children. The children with normal platelet count have a low recurrence rate and good prognosis, and those with a platelet count of <20×10/L have the worst prognosis.


Subject(s)
Child , Disease-Free Survival , Humans , Immunophenotyping , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Prognosis , Recurrence
8.
Article in Chinese | WPRIM | ID: wpr-775087

ABSTRACT

OBJECTIVE@#To study the long-term clinical effect of the CCLG-ALL2008 regimen in the treatment of children newly diagnosed with acute lymphoblastic leukemia (ALL) with different molecular biological features.@*METHODS@#A total of 940 children who were newly diagnosed with ALL were enrolled in this study. The children were treated with the CCLG-ALL2008 regimen. A retrospective analysis was performed for the long-term outcome of ALL children with different molecular biological features.@*RESULTS@#Among the 940 children with ALL, there were 570 boys and 370 girls, with a median age of onset of 5 years (range 1-15 years) and a median follow-up time of 65 months (range 3-123 months). The complete response (CR) rate was 96.7%, the predicted 10-year overall survival (OS) rate was 76.5%±1.5%, and the event-free survival (EFS) rate was 62.6%±3.0%. After CR was achieved after treatment, the overall recurrence rate was 21.9%. The children with positive ETV6-RUNX1 had the lowest recurrence rate and were prone to late recurrence, and those with positive MLL rearrangement had the highest recurrence rate and were prone to early recurrence. The children with positive ETV6-RUNX1 had a significantly higher predicted 10-year OS rate than those with positive TCF3-PBX1, BCR-ABL, or MLL rearrangement and those without molecular biological features (P<0.05). The children with positive ETV6-RUNX1 had a significantly higher predicted 10-year EFS rate than those with positive BCR-ABL or MLL rearrangement (P<0.05).@*CONCLUSIONS@#Molecular biological features may affect the long-term prognosis of children with ALL, and positive MLL rearrangement and BCR-ABL fusion gene are indicators of poor prognosis. Children with positive ETV6-RUNX1 fusion gene have the highest long-term survival rate.


Subject(s)
Adolescent , Antineoplastic Combined Chemotherapy Protocols , Therapeutic Uses , Child , Child, Preschool , Disease-Free Survival , Female , Fusion Proteins, bcr-abl , Humans , Infant , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Drug Therapy , Prognosis , Retrospective Studies
9.
Article in English | WPRIM | ID: wpr-691356

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the effects of ligustrazine (LTZ) on airway inflammation in a mouse model of neutrophilic asthma (NA).</p><p><b>METHODS</b>Forty healthy C57BL/6 female mice were randomly divided into 4 groups using a random number table, including the normal control, NA, LTZ and dexamethasone (DXM) groups, with 10 rats in each group. The NA mice model was established by the method of ovalbumin combined with lipopolysaccharide sensitization. At 0.5 h before each challenge, LTZ and DXM groups were intraperitoneally injected with LTZ (80 mg/kg) or DXM (0.5 mg/kg) for 14 d, respectively, while the other two groups were given the equal volume of normal saline. After last challenge for 24 h, the aerosol inhalation of methacholine was performed and the airway reactivity was measured. The bronchoalveolar lavage fluid (BALF) was collected. The Wright-Giemsa staining was used for total white blood cells and differential counts. The levels of cytokines interleukin (IL)-17 and IL-10 were detected by enzyme-linked immunosorbent assay. The pathological change of lung tissue was observed by hematoxylin eosin staining.</p><p><b>RESULTS</b>The airway responsiveness of the NA group was signifificantly higher than the normal control group (P<0.05), while those in the LTZ and DXM groups were signifificantly lower than the NA group (P<0.05). The neutrophil and eosinophil counts in the LTZ and DXM groups were signifificantly lower than the NA group (P<0.05), and those in the LTZ group were signifificantly lower than the DXM group (P<0.05). There were a large number of peribronchiolar and perivascular inflammatory cells in fifiltration in the NA group. The airway inflflammation in the LTZ and DXM groups were signifificantly alleviated than the NA group. The infifiltration in the LTZ group was signifificantly reduced than the DXM group. Compared with the normal control group, the IL-17 level in BALF was signifificantly increased and the IL-10 level in BALF was signifificantly decreased in the NA group (P<0.05). LTZ and DXM treatment signifificantly decreased IL-17 levels and increased IL-10 levels compared with the NA group (P<0.05), and the changes in the above indices were more signifificant in the LTZ group (P<0.05).</p><p><b>CONCLUSION</b>LTZ could alleviate the airway inflflammation in the NA mice model through increasing the IL-10 level and decreasing the IL-17 level.</p>


Subject(s)
Animals , Asthma , Blood , Drug Therapy , Pathology , Bronchoalveolar Lavage Fluid , Cell Biology , Disease Models, Animal , Female , Interleukin-10 , Metabolism , Interleukin-17 , Metabolism , Leukocyte Count , Lung , Pathology , Mice, Inbred C57BL , Neutrophils , Pathology , Pneumonia , Blood , Drug Therapy , Pathology , Pyrazines , Pharmacology , Therapeutic Uses , Respiratory Hypersensitivity , Blood , Drug Therapy , Pathology
10.
Article in Chinese | WPRIM | ID: wpr-261243

ABSTRACT

<p><b>OBJECTIVE</b>To identify the incidence of PAX5 deletion in childhood B-lineage acute lymphoblastic leukemia (B-ALL) without reproducible chromosomal abnormalities and to investigate the association between PAX5 abnormalities and prognosis of ALL.</p><p><b>METHODS</b>Multiplex ligation-dependent probe amplification was used to determine the copy numbers of PAX5 gene in children newly diagnosed with B-ALL without reproducible chromosomal abnormalities between April 2008 and April 2013 and controls (children with non-hematologic diseases or tumors). The patients were classifiied into deletion group and non-deletion group based on the presence of PAX5 deletion.</p><p><b>RESULTS</b>Eighteen (21%) out of 86 children with B-ALL had PAX5 deletion. The deletion group had a significantly higher total white blood cell count at diagnosis than the non-deletion group (P=0.001). The Kaplan-Meier analysis demonstrated that the deletion group had a significantly lower disease-free survival (DFS) rate than the non-deletion group (0.69±0.12 vs 0.90±0.04; P=0.017), but there was no significant difference in the overall survival rate between the two groups (P=0.128). The Cox analysis showed that PAX5 deletion was a risk factor for DFS (P=0.03).</p><p><b>CONCLUSIONS</b>PAX5 deletion is an independent risk factor for DFS in B-ALL children without reproducible chromosomal abnormalities.</p>


Subject(s)
Acute Disease , Adolescent , Cell Lineage , Child , Child, Preschool , Chromosome Aberrations , Disease-Free Survival , Female , Gene Deletion , Humans , Infant , Male , PAX5 Transcription Factor , Genetics , Precursor B-Cell Lymphoblastic Leukemia-Lymphoma , Genetics , Mortality
11.
Article in Chinese | WPRIM | ID: wpr-261155

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the association between clinical outcome and gene mutations in children with Fanconi anemia (FA).</p><p><b>METHODS</b>A retrospective analysis was performed for the clinical data of six children with the same severity of FA and receiving the same treatment. At first, single cell gel electrophoresis and chromosome breakage induced by mitomycin C were performed for diagnosis. Then the gene detection kit for congenital bone marrow failure diseases or complementation test was used for genotyping of FA. Finally the association between the clinical outcome at 3, 6, 9, or 12 months after treatment and gene mutation was analyzed.</p><p><b>RESULTS</b>Of all the six FA children, five had FANCA type disease, and one had FANCM type disease; four children carried two or more FA gene mutations. Among the children with the same severity of FA, those with more FA mutations had a younger age of onset and poorer response to medication, and tended to progress to a severe type.</p><p><b>CONCLUSIONS</b>Children carrying more than two FA mutations have a poor clinical outcome, and hematopoietic stem cell transplantation should be performed as soon as possible.</p>


Subject(s)
Child , Child, Preschool , Fanconi Anemia , Genetics , Female , Humans , Male , Mutation , Retrospective Studies
12.
Article in Chinese | WPRIM | ID: wpr-279901

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the application of multiplex ligation-dependent probe amplification (MLPA) in the detection of copy number variations (CNVs) in pediatric ETV6/RUNX1-positive acute lymphoblastic leukemia (ALL), to compare this method with conventional karyotype analysis and fluorescence in situ hybridization (FISH), and to evaluate the value of MLPA.</p><p><b>METHODS</b>The clinical data of 95 children with ETV6/RUNX1-positive ALL who were treated from January 2006 to November 2012 were analyzed retrospectively, including clinical features, results of karyotype analysis, and results of FISH. CNVs were detected with MLPA.</p><p><b>RESULTS</b>CNVs were detected in 73 (77%), and the median number of CNVs was 1 (range 0-6). The CNVs of EBF1, CDKN2A/2B, PAX5, ETV6, RB1, and BTG1 were detected in more than 10% of all the patients. The changes in the chromosome segments carrying the genes with CNVs detected by MLPA were not detected by conventional karyotype analysis. The coincidence rate between the CNVs in ETV6 gene detected by FISH and those detected by MLPA was 66%.</p><p><b>CONCLUSIONS</b>MLPA is an efficient and convenient method to detect CNVs in children with ETV6/RUNX1-positive ALL.</p>


Subject(s)
Adolescent , Child , Child, Preschool , Core Binding Factor Alpha 2 Subunit , DNA Copy Number Variations , Female , Humans , In Situ Hybridization, Fluorescence , Infant , Male , Multiplex Polymerase Chain Reaction , Methods , Oncogene Proteins, Fusion , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Genetics
13.
Article in Chinese | WPRIM | ID: wpr-289480

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the methylation rate of cyclin-dependent kinase inhibitor 2A (CDKN2A) and cyclin-dependent kinase inhibitor 2B (CDKN2B) in the 9P21 region in children with acute myeloid leukemia (AML) and the association of gene methylation with clinical features and outcomes.</p><p><b>METHODS</b>The clinical data of 58 children who were newly diagnosed with AML between January 2010 and December 2012 were retrospectively analyzed. Thirty-eight healthy children were recruited as the control group. Genomic DNA was extracted from bone marrow or peripheral blood of the 58 patients and 38 healthy children. The methylation status of CDKN2A and CDKN2B was analyzed by methylation-specific multiplex ligation-dependent probe amplification.</p><p><b>RESULTS</b>Gene methylation was not found in healthy children. Methylation probes of 44 patients were detected in 58 patients. The methylation of CDKN2A was detected with 136 bp and 237 bp methylation probes. The methylation of CDKN2B was detected with 130 bp, 210 bp, 220 bp, and 417 bp methylation probes. The methylation rate of CDKN2A was 5%, while the methylation rate of CDKN2B was 76%. The methylation detected by some probes was associated with sex, hemoglobin, and platelet count at the first visit.</p><p><b>CONCLUSIONS</b>The methylation of CDKN2B is a common event in children with AML, while the methylation of CDKN2A is relatively rare.</p>


Subject(s)
Adolescent , Child , Child, Preschool , Cyclin-Dependent Kinase Inhibitor p15 , Genetics , Cyclin-Dependent Kinase Inhibitor p16 , Genetics , DNA Methylation , Female , Humans , Infant , Leukemia, Myeloid, Acute , Genetics , Male
14.
Article in English | WPRIM | ID: wpr-293338

ABSTRACT

<p><b>OBJECTIVE</b>To observe the clinical effificacy of treatment with catgut implantation at acupoints on simple obesity.</p><p><b>METHODS</b>Following the theory of Chinese medicine (CM), pattern identification (PI) and treatment was based on the patient's symptoms and signs. Patients were observed during three courses and one year following treatment through self-comparison before and after six or seven acupoints catgut implantation. Obesity was divided into fifive types based on PI: (1) Stomach (Wei) and Intestine excess-heat, (2) Spleen (Pi) defificiency and phlegmwet stagnancy, (3) Liver (Gan)-qi stagnation, (4) Spleen-Kidney (Shen) yang deficiency, and (5) Liver-Kidney yin defificiency. Changes in the following measurements were recorded in 820 patients: body weight, body girth, skinfold thickness, body mass index (BMI), fat percentage (F%) and waist/hip ratio (WHR) and in the following blood values: leptin (LP), insulin (INS), blood lipids, fasting blood sugar (FBS), and insulin sensitive index (ISI) before and after the treatment. Values were compared with those of healthy controls (normal group).</p><p><b>RESULTS</b>Catgut implantation showed effificacy with all fifive types of obesity. Effificacy was greater in males than in females. There was no signifificant difference between the different types by Kruskal-Wallis H test, but the effect was best and of the highest number in patients with Stomach and Intestine excess-heat. Skin-fold thickness, body weight, waist circumference, F%, BMI, and WHR in all 820 cases decreased after treatment (at 90 days and one year), with signifificant differences before and after treatment (P<0.01). Improved metabolism of blood lipids was also seen. Following treatment, LP, INS, and FBS decreased signifificantly (P<0.01) and ISI increased signifificantly (P<0.05).</p><p><b>CONCLUSION</b>Catgut implantation at acupoints provided effective and persistent results, convenience, safety, painlessness, and prolonged effect with no side effects, resulting in reduced body weight and fat and improvement in body shape.</p>


Subject(s)
Acupuncture Points , Catgut , Female , Humans , Male , Obesity , Therapeutics , Weight Loss
15.
Article in Chinese | WPRIM | ID: wpr-346155

ABSTRACT

<p><b>OBJECTIVE</b>To study the clinical features of children with relapsed acute lymphoblastic leukemia (ALL) treated with the CCLG-ALL2008 protocol.</p><p><b>METHODS</b>The data of 591 children who were newly diagnosed with ALL and were treated with the CCLG-ALL 2008 protocol between April 2008 and June 2013 were collected, and the clinical features of 80 children with relapsed ALL were retrospectively analyzed.</p><p><b>RESULTS</b>After treatment with the CCLG-ALL2008 protocol, the recurrence rate in the standard-risk, intermediate-risk and the high-risk groups were 7.0%, 10.7% and 28.7% respectively (P<0.05). The recurrence rate in patients with TEL/AML1-positive ALL was 8.0%, and the 5-year overall survival (OS) of the relapsed patients was 37.04%. The recurrence rates in patients with MLL-positive and BCR/ABL-positive ALL were 35.0% and 24.2% respectively, and none of the relapsed patients had long-term survival. The recurrence mainly occurred at the very early stage (53%), and none of patients with recurrence at the very early stage had long-term survival. The recurrence occurred at early stage and late stage accounted for 34% and 14% respectively, and the 5-year OS rates of patients with recurrence at early stage and late stage were 11.44% and 60.00% respectively. The sites of recurrence were mainly bone marrow alone (83%), and the 5-year OS of patients with recurrence at bone marrow alone was 9.23%. The recurrence in bone marrow and outside bone marrow accounted for 11%, and the 5-year OS of patients with recurrence in both bone marrow and outside bone marrow was 25.00%. The recurrence only outside bone marrow accounted for 6%, and the 5-year OS of patients with recurrence only outside bone marrow was 100%. The recurrence rate in patients with T-cell ALL was 9.5%, and none of the relapsed patients had long-term survival. The recurrence rate in patients with B-cell ALL was 14.3%, and the 5-year OS of the relapsed patients was 15.52%.</p><p><b>CONCLUSIONS</b>After treatment with the CCLG-ALL2008 protocol, a relatively high recurrence rate is observed in children with high-risk ALL. Positive MLL and positive BCR/ABL are high-risk factors for recurrence. The recurrence rate is not significantly correlated with immunophenotype. A very low survival rate is seen in children whose recurrence have the following features: at early stage, only in bone marrow, T-cell ALL, and abnormal BCR/ABL and MLL.</p>


Subject(s)
Adolescent , Antineoplastic Combined Chemotherapy Protocols , Therapeutic Uses , Child , Child, Preschool , Female , Humans , Infant , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Drug Therapy , Mortality , Prognosis , Recurrence , Retrospective Studies
16.
Article in Chinese | WPRIM | ID: wpr-259596

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the copy number variations (CNV) of gene in pediatric acute myeloid leukemia (AML) and its correlation with clinical features and prognosis.</p><p><b>METHODS</b>The clinical data of 130 children aged <14 years with newly diagnosed AML from May 2006 to March 2013 were analyzed restrospectively. The CNV were analyzed by multiplex ligation-dependent probe amplification (MLPA). Thirty-eight normal children were selected in control group. All the data were statistically analyzed using SPSS16.0 software.</p><p><b>RESULTS</b>gene CNV of 2p24.3(MYCN), 10q23(PTEN) and 13q14(RB1, MIR15A, DLEU) were detected in more than 10% of the patients. CNV were detected in 49 cases(37.7%). The median loss and gain CNV frequencies per sample were 4. The CNV of TP53 correlated significantly with relapse. The loss ond gain CNV have no influence to EFS, DSF and OS. CNV were detected in the twelve percent of patients, but they were not detected with routine karyotype method.</p><p><b>CONCLUSION</b>The MLPA technique combined with karyotyping makes a substantial increase in the diagnostic rate. Patients with TP53 alterations have significantly higher relapse rate.</p>


Subject(s)
Child , DNA Copy Number Variations , Humans , Karyotype , Karyotyping , Leukemia, Myeloid, Acute , Multiplex Polymerase Chain Reaction , Prognosis
17.
Article in Chinese | WPRIM | ID: wpr-279045

ABSTRACT

<p><b>OBJECTIVE</b>To study the efficacy and safety of Chinese Childhood Leukemia Group ALL 2008 (CCLG-ALL2008) protocol combined with tyrosine kinase inhibitor (TKI, imatinib) for the treatment of Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) in children.</p><p><b>METHODS</b>The clinical data of 53 patients aged less than 15 years when first diagnosed with Ph+ ALL between October 2008 and December 2013 were retrospectively analyzed. The patients were assigned to two groups: HR (n=26) and HR+TKI (n=27). The HR group was treated with CCLG-ALL2008 protocol (for high-risk patients). The HR+TKI group was treated with imatinib in combination with CCLG-ALL2008 protocol (for high-risk patients).</p><p><b>RESULTS</b>The complete remission rate and chemotherapy induction-related mortality rate in the TKI+HR and HR groups were 100% vs 75% and 0 vs 15%, respectively. The 3-year event-free survival (EFS) rate in the HR group was (6±5)%; the 5-year EFS rate of the TKI+HR group was (52±11)%. Compared with the HR group, the TKI+HR group had no increase in the toxic responses to chemotherapy and had a decrease in the infection rate during the induction period.</p><p><b>CONCLUSIONS</b>Application of imatinib significantly improves the clinical efficacy in children with Ph+ ALL and has good safety.</p>


Subject(s)
Adolescent , Antineoplastic Agents , Therapeutic Uses , Child , Child, Preschool , Female , Humans , Imatinib Mesylate , Therapeutic Uses , Male , Philadelphia Chromosome , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Drug Therapy , Genetics , Mortality , Protein Kinase Inhibitors , Therapeutic Uses
18.
Article in Chinese | WPRIM | ID: wpr-279949

ABSTRACT

<p><b>OBJECTIVE</b>To identify IKZF1 gene copy number abnormalities in BCR/ABL-negative B-lineage acute lymphoblastic leukemia (B-ALL) in children, and to investigate the association between such abnormalities and prognosis.</p><p><b>METHODS</b>Multiplex ligation-dependent probe amplification (MLPA) was applied to detect IKZF1 gene copy number abnormalities in 180 children diagnosed with BCR/ABL-negative B-ALL. These children were classified into IKZF1 deletion group and IKZF1 normal group according to the presence or absence of IKZF1 gene deletion. The association between IKZF1 copy number abnormalities and prognosis of children with BCR/ABL-negative B-ALL was analyzed retrospectively.</p><p><b>RESULTS</b>Among 180 children, 27 (15.0%) had IKZF1 deletion; among the 27 children, 4 had complete deletions of 8 exons of IKZF1 gene, 17 had deletion of exon 1, 3 had deletions of exons 4-7, and 3 children had deletions of exons 2-7. Compared with those in the IKZF1 normal group, children in the IKZF1 deletion group had higher white blood cell (WBC) count and percentage of individuals with high risk of minimal residual disease at the first visit. IKZF1 deletions often occurred in BCR/ABL-negative children with no special fusion gene abnormalities. They were frequently accompanied by abnormalities in chromosomes 11, 8, 5, 7, and 21. The analysis with Kaplan-Meier method showed that disease-free survival (DFS) in the IKZF1 deletion group was significantly lower than that in the IKZF1 normal group (0.740 ± 0.096 vs 0.905 ± 0.034; P=0.002). Cox analysis showed that after exclusion of sex, age, initial WBC count, cerebrospinal fluid state at the first visit, prednisone response, and chromosome karyotype, IKZF1 deletion still affected the children's DFS (P<0.05).</p><p><b>CONCLUSIONS</b>Some children with BCR/ABL-negative B-ALL have IKZF1 deletion, and IKZF1 deletion is an independent risk factor for DFS in children with BCR/ABL-negative B-ALL.</p>


Subject(s)
Adolescent , Child , Child, Preschool , Female , Fusion Proteins, bcr-abl , Gene Dosage , Humans , Ikaros Transcription Factor , Genetics , Infant , Male , Multiplex Polymerase Chain Reaction , Precursor B-Cell Lymphoblastic Leukemia-Lymphoma , Genetics , Mortality , Prognosis
19.
Article in Chinese | WPRIM | ID: wpr-289543

ABSTRACT

<p><b>OBJECTIVE</b>To study the long-term efficacy of CAMSBDH-ALL chemotherapy protocol for the treatment of childhood acute lymphoblastic leukemia (ALL).</p><p><b>METHODS</b>Three hundred and eighteen children who were newly diagnosed with ALL between January 1999 and December 2007 were enrolled in this study. Among the 318 children, 83 children who hospitalized before December 2002 were treated with CAMSBDH-ALL99 protocol, including 48 patients of standard risk and 35 patients of high risk. The patients (n=235; 131 in standard risk and 104 in high risk) who hospitalized after December 2002 were treated with CAMSBDH-ALL03 protocol. Patients in the CAMSBDH-ALL99 protocol group were treated with conventional chemotherapy. CAMSBDH-ALL03 protocol was modified based on the CAMSBDH-ALL99 protocol.</p><p><b>RESULTS</b>The long-term overall survival (OS) and event-free-survival (EFS) in the CAMSBDH-ALL03 group was significantly higher than in the CAMSBDH-ALL99 (P<0.01). The long-term OS and EFS of standard risk and high risk patients in the CAMSBDH-ALL03 protocol group were significantly higher than in the CAMSBDH-ALL99 protocol group (P<0.01). The CAMSBDH-ALL03 protocol group showed a significantly lower recurrence rate (28.9%) than in the CAMSBDH-ALL99 protocol group (50.6%) (P<0.05). The mortality rate in the CAMSBDH-ALL03 protocol group was 28.5% vs 56.6% in the CAMSBDH-ALL99 protocol group (P<0.05).</p><p><b>CONCLUSIONS</b>The therapeutic effect of the CAMSBDH-ALL03 protocol is supior to the CAMSBDH-ALL99 protocol group for childhood ALL, with a higher long-term survival rate.</p>


Subject(s)
Adolescent , Antineoplastic Combined Chemotherapy Protocols , Therapeutic Uses , Child , Child, Preschool , Clinical Protocols , Female , Humans , Infant , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Drug Therapy , Mortality , Recurrence
20.
Article in Chinese | WPRIM | ID: wpr-254237

ABSTRACT

<p><b>OBJECTIVE</b>To determine the expression level of silent mating-type information regulation 2 homologue 1 (SIRT1) in bone marrow biopsy tissues among children with acute myeloid leukemia (AML) and analyze its relationship with the prognosis of AML patients.</p><p><b>METHODS</b>A retrospective analysis was performed on the clinical data of 54 children who were diagnosed with AML between July 2009 and April 2012 and who underwent bone marrow biopsy at diagnosis. The expression of SIRT1 in bone marrow was measured by immunohistochemistry. The 54 patients were divided into two groups according to the expression of SIRT1: SIRT1-negative (n=10) and SIRT1-positive (n=44). The SIRT1-positive group was further divided into three subgroups: SIRT1(+) (n=8), SIRT1(++) (n=7) and SIRT1(+++) (n=29) according to the expression levels of SIRT1. Cox multivariate regression analysis was used to determine the unfavorable factors for long survival in children with AML.</p><p><b>RESULTS</b>The SIRT1(+++) subgroup had a significantly higher mortality than the SIRT1-negative group (P<0.05). Compared with the SIRT1-negative group, the SIRT1-positive group had a significantly lower 2-year overall survival rate (P<0.05) and a significantly lower 2-year event-free survival rate (P<0.05). Cox multivariate regression analysis showed that positive expression of SIRT1 was an unfavorable factor for long-term survival in children with AML, with a risk coefficient of 2.071 (95% CI: 1.017-4.219; P=0.045).</p><p><b>CONCLUSIONS</b>SIRT1 is overexpressed in some of pediatric AML patients, and the overexpression of SIRT1 is associated with poor prognosis.</p>


Subject(s)
Adolescent , Biopsy , Bone Marrow , Chemistry , Pathology , Child , Child, Preschool , Female , Humans , Leukemia, Myeloid, Acute , Metabolism , Mortality , Male , Proportional Hazards Models , Retrospective Studies , Sirtuin 1 , Survival Rate
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