BACKGROUND Myelodysplastic syndromes(MDSs)are a group of hematological diseases caused by expansion of an abnormal clone of hematopoietic stem cells.Primary MDS is a potentially premalignant clonal disorder that may p...BACKGROUND Myelodysplastic syndromes(MDSs)are a group of hematological diseases caused by expansion of an abnormal clone of hematopoietic stem cells.Primary MDS is a potentially premalignant clonal disorder that may progress to overt acute leukemia in 25%-50%of cases.However,most of these cases evolve into acute myeloid leukemia and rarely progress to acute lymphoblastic leukemia(ALL).Thus,transformation of MDS into B-cell ALL is rare.CASE SUMMARY A 58-year-old man was admitted to the hospital for reduced blood cell counts.Based on all the test results and the World Health Organization diagnosis and classification,the patient was finally diagnosed with ring-shaped sideroblastic MDS with refractory hemocytopenia due to multilineage dysplasia.We used red blood cell transfusions and other symptomatic support treatments.After 4 years,the patient felt dizziness,fatigue,and night sweats.We improved bone marrow and peripheral blood and other related auxiliary examinations.He was eventually diagnosed with B-lineage acute lymphocytic leukemia(MDS transformation).CONCLUSION The number of peripheral blood cells,type of MDS,proportion of primitive cells in bone marrow,and number and quality of karyotypes are all closely related to the conversion of MDS to ALL.展开更多
Objective:Almost all leukemia patients died,fortunately now with the advancement of medicine and science and with the advent of treatment Chemotherapy,Radiotherapy,and Surgical treatment 75%of patients with leukemia w...Objective:Almost all leukemia patients died,fortunately now with the advancement of medicine and science and with the advent of treatment Chemotherapy,Radiotherapy,and Surgical treatment 75%of patients with leukemia who need treatment for up to 5 years,live longer than patients with leukemia who do not receive treatment.On the one side,in our country,Afghanistan the number of incidents has increased recently,and on the other side,we do not have data at the national level.Therefore,the child health hospital administration considered the necessary and left me to research this case in the last 6 months of 2018.Methods:This is an observational descriptive study,which observed 10,293 patients that come to the pediatric internal which 300 patients who indicate leukemia,and 200 patients with acute lymphoblastic leukemia(ALL),who were admitted to the oncology service of Andhra Gandhi Child Health Hospital in 2018 the study took place.Results:Based on the age we detect less than one year’s 10(5%)patients,1-10 years old 150(75%)patients,and more than 10 years old 40(20%)patients.Based on sex,boys were 120(60%)patients and girls 80(40%)patients.Based on clinical findings,anemia 155(77.5%)patients,fever 130(65%)patients,bleeding 90(45%)patients,spleen thickness 88(44%)patients,liver thickness 73(36.6%)patients,lymph nodes thickness 70(35.55%)patients,great pains 66(33%)patients and nervous system disorders 38(19%)patients.Conclusion:We can say with great conviction that 200 patients out of 10,293 had acute lymphoblastic leukemia.Further studies with prospective nature are required to confirm this observation.展开更多
Objective： This study aimed to express a fusion protein of diphtheria toxin and human B cell-activating factor （DT388sBAFF） in Escherichia coli （E. coli） and investigate its activity in human B-lineage acute lymp...Objective： This study aimed to express a fusion protein of diphtheria toxin and human B cell-activating factor （DT388sBAFF） in Escherichia coli （E. coli） and investigate its activity in human B-lineage acute lymphoblastic leukemia 1 cells （BALL-1）. Methods： A fragment of DT388sBAFF fusion gene was separated from plasmid pUC57-DT388sBAFF digested with Nde I and Xho I, and inserted into the expression vector pcold II digested with the same enzymes. Recombinants were screened by the colony polymerase chain reaction （PCR） and restriction map. The recombinant expression vector was transformed into BL21 and its expression was induced by isopropyl β-D-1-thiogalactopyranoside （IPTG）. The recombinant protein was identified by sodium dodecyl sulfate-polyacrylamide gel electrophoresis （SDS-PAGE） and Western blot, and then purified by Ni2＋-NTA affinity chromatography. The expression level of B cell-activating factor receptor （BAFF-R） on BALL-1 cells was assessed by real-time PCR. The receptor binding capacity of recombinant protein was determined by cell fluorescent assay. The specific cytotoxicity of recombinant protein on BALL-1 cells was detected by 3-（4,5-dimethylthiazolyl-2）-2,5-diphenyltetrazolium bromide （MTT） assay. Results： The expression level of recombinant protein was 50% of total bacterial proteins in E. coli, and the recombinant protein could bind to BAFF-R-positive BALL-1 cells and thereby produce a cytotoxic effect on the cells. Conclusion： The fusion protein expression vector DT388sBAFF was successfully constructed and the recombinant protein with selective cytotoxicity against BALL-1 cells was obtained, providing foundation for further study of the therapy of human B-lineage acute lymphoblastic leukemia.展开更多
Background: Obesity in pediatric ALL survivors is a well recognized late effect. Hence the present study examines the BMI-for-age of Indian childhood ALL and NHL survivors. Method: A retrospective study of 118 ALL/NHL...Background: Obesity in pediatric ALL survivors is a well recognized late effect. Hence the present study examines the BMI-for-age of Indian childhood ALL and NHL survivors. Method: A retrospective study of 118 ALL/NHL survivors and 138 age sex matched was carried out. From the recorded heights and weights were body mass index (BMI) was computed. The survivor data was compared with 138 controls from the data set collected by investigators previously. Results: 82.8% of patients had BMI-for-age in 5th-84th percentile (healthy) at time of diagnosis and at inclusion in the study. Comparison of BMI of survivors with matched controls was not significant. However, The mean BMI-for-age for younger patients (3 to 12 years) was significantly higher than mean BMI-for-age of matched controls. Distribution of data by time elapsed from therapy was significant. Overweight/obesity was observed among the survivors who were off therapy for two years with increase in after four years post-therapy. Conclusion: Our preliminary study indicates late effects of therapy and points to the need of long term assessment of the survivors, even though majority of them were within the normal weight range.展开更多
BACKGROUNDβ-thalassemia intermedia(βTI)is one of the hemoglobinopathies.It constitutes 10%ofβ-thalassemia cases yet being associated with a better quality of life thanβ-thalassemia major(βTM).CASE SUMMARY We rece...BACKGROUNDβ-thalassemia intermedia(βTI)is one of the hemoglobinopathies.It constitutes 10%ofβ-thalassemia cases yet being associated with a better quality of life thanβ-thalassemia major(βTM).CASE SUMMARY We recently reported the first case of acute lymphoblastic leukemia(ALL)from Egypt in a child withβTM,and we herein report the first case of ALL from Egypt in a child withβTI.In this report,literature was reviewed for cases of malignancies associated withβTI and the possible factors underling the relationship between the two entities.CONCLUSION We stress that physicians should have a high index of suspicion of malignancies in thalassemia patients if they present with any suggestive symptoms or signs.展开更多
Background: The treatment used to combat acute lymphoblastic leukemia (ALL) is multidrug;therefore it is important to use active pharmacovigilance to detect, assess and analyze the likely adverse reactions which may o...Background: The treatment used to combat acute lymphoblastic leukemia (ALL) is multidrug;therefore it is important to use active pharmacovigilance to detect, assess and analyze the likely adverse reactions which may occur during the same period. Objective: To determine the frequency of adverse reactions to chemotherapeutic drugs in children with ALL. Material and Methods: Intensive pharmacovigilance was used to record the reports of adverse reactions to vincristine, L-asparaginase and the vincristine-L-asparaginase combination in children with ALL in a paediatric hospital. For each notification, the adverse reactions were analyzed in order to verify causality. Results: Forty patients were evaluated. Twenty children were female (50.0%) and 20 were male (50%). The children had a mean age, weight and height (±standard deviation: SD) of 8.1 (±3.4) years, 31.4 (±13.9) kg and 1.3 (±0.2) m, respectively. Vincristine was administered to 19 patients, vincristine plus L-asparaginase were given to 19 patients and only 2 patients used L-asparaginase. One-hundred-ninety adverse reactions were detected in the patients, with an average (±SD) of 4.8 (±2.6). Ondansetron was the drug administered for the treating of nausea and vomiting. One hundred eighty-one (95.3%) adverse reactions were identified as “definite”, 5 (2.6%) as “probable” and 4 (2.1%) as “doubtful”. Conclusions: There is a high incidence of adverse reactions by the administration of vincristine and L-asparaginase;the reactions of highest incidence were: nausea, vomiting, neutropenia, diarrhea, constipation, mucositis, headache, and abdominal pain. It is important to promote the detection, collection, reporting, assessment and treatment of ARD’s in children. It is necessary to promote the conduct further studies on pharmacovigilance with this type of treatments and to increase the duration of the studies.展开更多
Background: The role of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children with high-risk (HR) T-cell acute lymphoblastic leukemia (T-ALL) in first complete remission (CR1) is still under evalu...Background: The role of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children with high-risk (HR) T-cell acute lymphoblastic leukemia (T-ALL) in first complete remission (CR1) is still under evaluation. Moreover, relapse is the main factor affecting survival. This study aimed to explore the effect of allo-HSCT (especially haploidentical HSCT [haplo-HSCT]) on improving survival and reducing relapse for HR childhood T-ALL in CR1 and the prognostic factors of childhood T-ALL in order to identify who could benefit from HSCT.Methods: A total of 74 newly diagnosed pediatric T-ALL patients between January 1, 2012 and June 30, 2018 were enrolled in this retrospective study. Patients were stratified into the low-risk chemotherapy cohort (n = 16), HR chemotherapy cohort (n = 31), and HR transplant cohort (n = 27). Characteristics, survival outcomes, and prognostic factors of all patients were then analyzed.Results: Patient prognosis in the HR chemotherapy cohort was significantly worse than that in the low-risk chemotherapy cohort (5-year overall survival [OS]: 58.5%vs. 100%,P = 0.003;5-year event-free survival [EFS]: 54.1%vs. 83.4%,P = 0.010;5-year cumulative incidence of relapse [CIR]: 45.2%vs. 6.3%,P = 0.011). In HR patients, allo-HSCT improved the 5-year EFS and CIR compared to that of chemotherapy (5-year EFS: 80.1%vs. 54.1%,P = 0.041;5-year CIR: 11.6%vs. 45.2%,P = 0.006). The 5-year OS was higher in the HR transplant cohort than that in the HR chemotherapy cohort (81.0%vs. 58.5%,P = 0.084). Minimal residual disease re-emergence was an independent risk factor for 5-year OS, EFS, and CIR;age ≥10 years was an independent risk factor for OS and EFS;and high white blood cell count was an independent risk factor for EFS and CIR.Conclusion: Allo-HSCT, especially haplo-HSCT, could effectively reduce relapse of children with HR T-ALL in CR1.展开更多
目的分析儿童急性淋巴细胞白血病(acute lymphoblastic leukemia,ALL)经中国儿童肿瘤协作组急性淋巴细胞白血病2015方案(Chinese Children's Cancer Group ALL-2015 protocol,CCCG-ALL-2015)治疗后的累积复发率(cumulative incidenc...目的分析儿童急性淋巴细胞白血病(acute lymphoblastic leukemia,ALL)经中国儿童肿瘤协作组急性淋巴细胞白血病2015方案(Chinese Children's Cancer Group ALL-2015 protocol,CCCG-ALL-2015)治疗后的累积复发率(cumulative incidence of relapse,CIR),并探讨影响复发的危险因素。方法回顾性分析2015年1月—2019年12月接受CCCG-ALL-2015方案治疗的852例患儿的临床资料,计算CIR并分析影响儿童急性B淋巴细胞白血病(B-ALL)复发的危险因素。结果852例ALL患儿中,146例(17.1%)发生复发,8年CIR为(19.8±1.6)%。B-ALL与急性T淋巴细胞白血病患儿的8年CIR比较差异无统计学意义(P>0.05)。146例复发患儿中,复发时间主要集中于极早期(62例,42.5%)和早期(46例,31.5%),极早期单纯骨髓复发42例(28.8%),早期单纯骨髓复发27例(18.5%)。Cox比例风险回归模型分析显示,融合基因MLLr阳性(HR=4.177,95%CI:2.086~8.364,P<0.001)和第46天微小残留病≥0.01%(HR=2.013,95%CI:1.163~3.483,P=0.012)是B-ALL患儿经CCCG-ALL-2015方案治疗后复发的危险因素。结论儿童ALL经CCCG-ALL-2015方案治疗后仍有较高的复发率,以极早期和早期单纯骨髓复发常见;第46天微小残留病≥0.01%、融合基因MLLr阳性与B-ALL复发密切相关。展开更多
基金Shandong Medicine and Health Science Technology Development Plan,No.2017WSA08039Jining Key Research and Development Program,No.2018SMNS008+1 种基金Doctoral Research Startup Foundation of Jining First People’s Hospital,No.2019003and TCM Technology Development Plan,No.20190744.
文摘BACKGROUND Myelodysplastic syndromes(MDSs)are a group of hematological diseases caused by expansion of an abnormal clone of hematopoietic stem cells.Primary MDS is a potentially premalignant clonal disorder that may progress to overt acute leukemia in 25%-50%of cases.However,most of these cases evolve into acute myeloid leukemia and rarely progress to acute lymphoblastic leukemia(ALL).Thus,transformation of MDS into B-cell ALL is rare.CASE SUMMARY A 58-year-old man was admitted to the hospital for reduced blood cell counts.Based on all the test results and the World Health Organization diagnosis and classification,the patient was finally diagnosed with ring-shaped sideroblastic MDS with refractory hemocytopenia due to multilineage dysplasia.We used red blood cell transfusions and other symptomatic support treatments.After 4 years,the patient felt dizziness,fatigue,and night sweats.We improved bone marrow and peripheral blood and other related auxiliary examinations.He was eventually diagnosed with B-lineage acute lymphocytic leukemia(MDS transformation).CONCLUSION The number of peripheral blood cells,type of MDS,proportion of primitive cells in bone marrow,and number and quality of karyotypes are all closely related to the conversion of MDS to ALL.
文摘Objective:Almost all leukemia patients died,fortunately now with the advancement of medicine and science and with the advent of treatment Chemotherapy,Radiotherapy,and Surgical treatment 75%of patients with leukemia who need treatment for up to 5 years,live longer than patients with leukemia who do not receive treatment.On the one side,in our country,Afghanistan the number of incidents has increased recently,and on the other side,we do not have data at the national level.Therefore,the child health hospital administration considered the necessary and left me to research this case in the last 6 months of 2018.Methods:This is an observational descriptive study,which observed 10,293 patients that come to the pediatric internal which 300 patients who indicate leukemia,and 200 patients with acute lymphoblastic leukemia(ALL),who were admitted to the oncology service of Andhra Gandhi Child Health Hospital in 2018 the study took place.Results:Based on the age we detect less than one year’s 10(5%)patients,1-10 years old 150(75%)patients,and more than 10 years old 40(20%)patients.Based on sex,boys were 120(60%)patients and girls 80(40%)patients.Based on clinical findings,anemia 155(77.5%)patients,fever 130(65%)patients,bleeding 90(45%)patients,spleen thickness 88(44%)patients,liver thickness 73(36.6%)patients,lymph nodes thickness 70(35.55%)patients,great pains 66(33%)patients and nervous system disorders 38(19%)patients.Conclusion:We can say with great conviction that 200 patients out of 10,293 had acute lymphoblastic leukemia.Further studies with prospective nature are required to confirm this observation.
基金supported by grants from the National "973" Basic Research Program of China (No.2012CB944703)the National Key Technology Research and Development Program of China (No.2011BAI17B00)the Shandong Provincial Science and Technology Development Projects (No.2009GG10002008 and No.2011GSF12103)
文摘Objective： This study aimed to express a fusion protein of diphtheria toxin and human B cell-activating factor （DT388sBAFF） in Escherichia coli （E. coli） and investigate its activity in human B-lineage acute lymphoblastic leukemia 1 cells （BALL-1）. Methods： A fragment of DT388sBAFF fusion gene was separated from plasmid pUC57-DT388sBAFF digested with Nde I and Xho I, and inserted into the expression vector pcold II digested with the same enzymes. Recombinants were screened by the colony polymerase chain reaction （PCR） and restriction map. The recombinant expression vector was transformed into BL21 and its expression was induced by isopropyl β-D-1-thiogalactopyranoside （IPTG）. The recombinant protein was identified by sodium dodecyl sulfate-polyacrylamide gel electrophoresis （SDS-PAGE） and Western blot, and then purified by Ni2＋-NTA affinity chromatography. The expression level of B cell-activating factor receptor （BAFF-R） on BALL-1 cells was assessed by real-time PCR. The receptor binding capacity of recombinant protein was determined by cell fluorescent assay. The specific cytotoxicity of recombinant protein on BALL-1 cells was detected by 3-（4,5-dimethylthiazolyl-2）-2,5-diphenyltetrazolium bromide （MTT） assay. Results： The expression level of recombinant protein was 50% of total bacterial proteins in E. coli, and the recombinant protein could bind to BAFF-R-positive BALL-1 cells and thereby produce a cytotoxic effect on the cells. Conclusion： The fusion protein expression vector DT388sBAFF was successfully constructed and the recombinant protein with selective cytotoxicity against BALL-1 cells was obtained, providing foundation for further study of the therapy of human B-lineage acute lymphoblastic leukemia.
文摘Background: Obesity in pediatric ALL survivors is a well recognized late effect. Hence the present study examines the BMI-for-age of Indian childhood ALL and NHL survivors. Method: A retrospective study of 118 ALL/NHL survivors and 138 age sex matched was carried out. From the recorded heights and weights were body mass index (BMI) was computed. The survivor data was compared with 138 controls from the data set collected by investigators previously. Results: 82.8% of patients had BMI-for-age in 5th-84th percentile (healthy) at time of diagnosis and at inclusion in the study. Comparison of BMI of survivors with matched controls was not significant. However, The mean BMI-for-age for younger patients (3 to 12 years) was significantly higher than mean BMI-for-age of matched controls. Distribution of data by time elapsed from therapy was significant. Overweight/obesity was observed among the survivors who were off therapy for two years with increase in after four years post-therapy. Conclusion: Our preliminary study indicates late effects of therapy and points to the need of long term assessment of the survivors, even though majority of them were within the normal weight range.
文摘BACKGROUNDβ-thalassemia intermedia(βTI)is one of the hemoglobinopathies.It constitutes 10%ofβ-thalassemia cases yet being associated with a better quality of life thanβ-thalassemia major(βTM).CASE SUMMARY We recently reported the first case of acute lymphoblastic leukemia(ALL)from Egypt in a child withβTM,and we herein report the first case of ALL from Egypt in a child withβTI.In this report,literature was reviewed for cases of malignancies associated withβTI and the possible factors underling the relationship between the two entities.CONCLUSION We stress that physicians should have a high index of suspicion of malignancies in thalassemia patients if they present with any suggestive symptoms or signs.
文摘Background: The treatment used to combat acute lymphoblastic leukemia (ALL) is multidrug;therefore it is important to use active pharmacovigilance to detect, assess and analyze the likely adverse reactions which may occur during the same period. Objective: To determine the frequency of adverse reactions to chemotherapeutic drugs in children with ALL. Material and Methods: Intensive pharmacovigilance was used to record the reports of adverse reactions to vincristine, L-asparaginase and the vincristine-L-asparaginase combination in children with ALL in a paediatric hospital. For each notification, the adverse reactions were analyzed in order to verify causality. Results: Forty patients were evaluated. Twenty children were female (50.0%) and 20 were male (50%). The children had a mean age, weight and height (±standard deviation: SD) of 8.1 (±3.4) years, 31.4 (±13.9) kg and 1.3 (±0.2) m, respectively. Vincristine was administered to 19 patients, vincristine plus L-asparaginase were given to 19 patients and only 2 patients used L-asparaginase. One-hundred-ninety adverse reactions were detected in the patients, with an average (±SD) of 4.8 (±2.6). Ondansetron was the drug administered for the treating of nausea and vomiting. One hundred eighty-one (95.3%) adverse reactions were identified as “definite”, 5 (2.6%) as “probable” and 4 (2.1%) as “doubtful”. Conclusions: There is a high incidence of adverse reactions by the administration of vincristine and L-asparaginase;the reactions of highest incidence were: nausea, vomiting, neutropenia, diarrhea, constipation, mucositis, headache, and abdominal pain. It is important to promote the detection, collection, reporting, assessment and treatment of ARD’s in children. It is necessary to promote the conduct further studies on pharmacovigilance with this type of treatments and to increase the duration of the studies.
基金2018 Beijing Municipal Key Clinical Specialty Construction Project-Pediatrics(No. 2199000726)。
文摘Background: The role of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children with high-risk (HR) T-cell acute lymphoblastic leukemia (T-ALL) in first complete remission (CR1) is still under evaluation. Moreover, relapse is the main factor affecting survival. This study aimed to explore the effect of allo-HSCT (especially haploidentical HSCT [haplo-HSCT]) on improving survival and reducing relapse for HR childhood T-ALL in CR1 and the prognostic factors of childhood T-ALL in order to identify who could benefit from HSCT.Methods: A total of 74 newly diagnosed pediatric T-ALL patients between January 1, 2012 and June 30, 2018 were enrolled in this retrospective study. Patients were stratified into the low-risk chemotherapy cohort (n = 16), HR chemotherapy cohort (n = 31), and HR transplant cohort (n = 27). Characteristics, survival outcomes, and prognostic factors of all patients were then analyzed.Results: Patient prognosis in the HR chemotherapy cohort was significantly worse than that in the low-risk chemotherapy cohort (5-year overall survival [OS]: 58.5%vs. 100%,P = 0.003;5-year event-free survival [EFS]: 54.1%vs. 83.4%,P = 0.010;5-year cumulative incidence of relapse [CIR]: 45.2%vs. 6.3%,P = 0.011). In HR patients, allo-HSCT improved the 5-year EFS and CIR compared to that of chemotherapy (5-year EFS: 80.1%vs. 54.1%,P = 0.041;5-year CIR: 11.6%vs. 45.2%,P = 0.006). The 5-year OS was higher in the HR transplant cohort than that in the HR chemotherapy cohort (81.0%vs. 58.5%,P = 0.084). Minimal residual disease re-emergence was an independent risk factor for 5-year OS, EFS, and CIR;age ≥10 years was an independent risk factor for OS and EFS;and high white blood cell count was an independent risk factor for EFS and CIR.Conclusion: Allo-HSCT, especially haplo-HSCT, could effectively reduce relapse of children with HR T-ALL in CR1.
文摘目的分析儿童急性淋巴细胞白血病(acute lymphoblastic leukemia,ALL)经中国儿童肿瘤协作组急性淋巴细胞白血病2015方案(Chinese Children's Cancer Group ALL-2015 protocol,CCCG-ALL-2015)治疗后的累积复发率(cumulative incidence of relapse,CIR),并探讨影响复发的危险因素。方法回顾性分析2015年1月—2019年12月接受CCCG-ALL-2015方案治疗的852例患儿的临床资料,计算CIR并分析影响儿童急性B淋巴细胞白血病(B-ALL)复发的危险因素。结果852例ALL患儿中,146例(17.1%)发生复发,8年CIR为(19.8±1.6)%。B-ALL与急性T淋巴细胞白血病患儿的8年CIR比较差异无统计学意义(P>0.05)。146例复发患儿中,复发时间主要集中于极早期(62例,42.5%)和早期(46例,31.5%),极早期单纯骨髓复发42例(28.8%),早期单纯骨髓复发27例(18.5%)。Cox比例风险回归模型分析显示,融合基因MLLr阳性(HR=4.177,95%CI:2.086~8.364,P<0.001)和第46天微小残留病≥0.01%(HR=2.013,95%CI:1.163~3.483,P=0.012)是B-ALL患儿经CCCG-ALL-2015方案治疗后复发的危险因素。结论儿童ALL经CCCG-ALL-2015方案治疗后仍有较高的复发率,以极早期和早期单纯骨髓复发常见;第46天微小残留病≥0.01%、融合基因MLLr阳性与B-ALL复发密切相关。
