Therapeutic options for gastric variceal bleeding in the presence of extensive portal vein thrombosis associated with a myeloproliferative disorder are limited.We report a case of a young woman who presented with gast...Therapeutic options for gastric variceal bleeding in the presence of extensive portal vein thrombosis associated with a myeloproliferative disorder are limited.We report a case of a young woman who presented with gastric variceal bleeding secondary to extensive splanchnic venous thrombosis due to a Janus kinase 2 mutation associated myeloproliferative disorder that was managed effectively with partial splenic embolization.展开更多
This experiment was designed to study about the regulation and mechanisms of Buzhong Yiqi-Pill on bone marrow hema topoietic system.Bone marrow inhibition of mouse model was established to simulate blood deficiency sy...This experiment was designed to study about the regulation and mechanisms of Buzhong Yiqi-Pill on bone marrow hema topoietic system.Bone marrow inhibition of mouse model was established to simulate blood deficiency syndrome.Semi-solid agar culture assay of hematopoietic progenitor cells(HPC)in vitro and flow cytometry were used.To study the effect of Buzhong Yiqi-Pill on bone marrow hematopoietic function of myelosuppressed mice,HPC survival,colony forming rate,bone marrow call cycle and cell apoptosis was detected.展开更多
This study was to determine whether GM-CSF induced WT1 gene expression and to establish an association with markers of proliferation CD71+CD34+ using nPCR and flow cytometry respectively, in samples obtained from 5 ne...This study was to determine whether GM-CSF induced WT1 gene expression and to establish an association with markers of proliferation CD71+CD34+ using nPCR and flow cytometry respectively, in samples obtained from 5 newly diagnosed JMML patients. Overtime (day 0 to day 14) there was an insignificant difference in WT1 gene expression and CD71+CD34+ in JMML samples when compared to peripheral blood of normal volunteers (n = 3). Our study suggests that there is a correlation between WT1 gene expression and cellular proliferation and that GMCSF in vitro does not create a significant difference in JMML samples.展开更多
To the Editor:Allogeneic hemopoietic stem cell transplantation(allo-HSCT)represents the only long-term survival treatment choice for patients with myelodysplastic syndromes(MDS)and MDS/myeloproliferative neoplasm(MPN)...To the Editor:Allogeneic hemopoietic stem cell transplantation(allo-HSCT)represents the only long-term survival treatment choice for patients with myelodysplastic syndromes(MDS)and MDS/myeloproliferative neoplasm(MPN).Unfortunately,post-hemopoietic stem cell transplantation(HSCT)relapse remains a cause of treatment failure and the results of salvage treatments are poor.Developing better conditioning regimens is urgently needed.Previous studies have shown synergistic antileukemic effects between decitabine(DEC)and idarubicin(IDA).[1]In an attempt to design a conditioning strategy with very low toxicity but considerable myelosuppressive activity and potential immune-enhancing effects for patients with high-risk MDS and MDS/MPN,we combined DEC and IDA with busulfan,cyclophosphamide,andudarabine for a modied myeloablative regimen in this prospective,multicenter cohort study(hereafter referred to as the“DEC/IDA study”).展开更多
In the treatment of tumor patients introduction of multidrug resistance genes into hematopoietic cells has been reported as an approach for reducing myelotoxicity created by antitumor drugs. However, the nonspecific e...In the treatment of tumor patients introduction of multidrug resistance genes into hematopoietic cells has been reported as an approach for reducing myelotoxicity created by antitumor drugs. However, the nonspecific expression of the genes can also increase the chemoresistance of the tumor cells invaded into bone marrow, which influences seriously the effectiveness of chemotherapy. In this study, a new strategy is described for specific myeloprotection. The recombinant retroviral vector containing multidrug resistance 1 (MDR1) gene regulated by aminopeptidase N (APN) myeloid promoter was constructed and then introduced into myeloblastic cells KGla and tumor cell line BEL7402. The specific transcript of MDR1 was detected in KGla cells transduced with MDR1 gene and rhodamine 123 was effectively extruded by Pgp, the protein of MDR1 gene. The resistance elevated markedly by 10.6, 10.4, 11.2, 4.2 and 14.2 folds in MDR1 gene-transduced KGla cells to chemothera-peutic drugs such as cochicine, VP-16,展开更多
In a recently published study in The New England Journal of Medicine,Liu and colleagues were able to demonstrate that-apart from the antileukemic effects previously reported for hypomethylating agents(HMAs)-treatment ...In a recently published study in The New England Journal of Medicine,Liu and colleagues were able to demonstrate that-apart from the antileukemic effects previously reported for hypomethylating agents(HMAs)-treatment with these drugs can also lead to demethylation and subsequent upregulation of oncogenes resulting in shorter survival in patients with myelo-dysplastic syndrome(MDS).展开更多
Objective To detect the expression level of growth differentiation factor 11(GDF11)in patients with myelodysplastic syndrome(MDS),and to evaluate the relationship between GDF11 level and erythropoiesis functions.Metho...Objective To detect the expression level of growth differentiation factor 11(GDF11)in patients with myelodysplastic syndrome(MDS),and to evaluate the relationship between GDF11 level and erythropoiesis functions.Methods A total of 44 MDS patients(18 low-risk group patients and 26 high-risk group patients)in the Department of Hematology in Tianjin Medical展开更多
Objective To assess the efficacy and toxicity of decitabine-based conditioning regimen in patients with myelodysplastic syndrome(MDS),acute myeloid leukemia secondary to MDS(MDS-AML)or chronic myelomonocytic leukemia(...Objective To assess the efficacy and toxicity of decitabine-based conditioning regimen in patients with myelodysplastic syndrome(MDS),acute myeloid leukemia secondary to MDS(MDS-AML)or chronic myelomonocytic leukemia(CMML).Methods From March 1,2013 to May 25,2015,22 patients who underwent allogenic hematopoietic stem cell transplantation(allo-HSCT)with decitabine-based conditioning regimen were analyzed retrospectively.Results①22 patients,14 males and 8 females with a median age of 42.5(24-56)years old,were diagnosed as MDS(n=14),CMML(n=4),MDS-AML(n=4).②15 patients were treated with the conditioning regimen of decitabine combined with busulfan,cyclophosphamide,fludarabine,and cytarabine,the other 7 cases were treated with decitabine,busulfan,fludarabine,and cytarabine.The dose of decitabine was 20 mg·m^-2·d^-1 for 5 days.Rabbit anti-human anti-thymocyte globulin(2.5 mg·kg^-1·d^-1 for 4 days)was involved in conditioning regimen in patients with unrelated donor or haploidentical transplantation.③Except 1 patient died of infection in 2 months after transplantation,the other patients were engrafted successfully.The median time of granulocyte engraftment was 13(12-18)days,and the median time of platelet engraftment was 16(13-81)days.④The incidence of acute graft versus host disease(aGVHD)was(41.3±10.6)%,and severe aGVHD(grade ofⅢ-Ⅳ)was(18.4±9.7)%.The incidence of chronic graft versus host disease(cGVHD)was(56.4±11.3)%,and extensive cGVHD was(36.4±12.1)%.⑤8 patients were suffered with cytomegalovirus(CMV)viremia.Among the 18 patients with definite infection,6 occurred during myelosuppression and 12 cases occurred after hematopoietic reconstruction.The 2-year and 3-year nonrelapse mortality was(13.9±7.4)%and(24.3±9.5)%,respectively.⑥The 2-year and 3-year overallsurvival(OS)was(77.3±8.9)%and(67.9±10.0)%,respectively.The 2-year and 3-year relapsefreesurvival(RFS)was(72.7±9.5)%and(63.6±10.3)%,respectively.Conclusion allo-HSCT withdecitabine-based conditioning regimen is feasible in thetreatment of MDS,MDS-AML or CMML.展开更多
Objective To explore the outcome of cyclosporine A(CsA) combined with danazol with or without thalidomide regimen for myelodysplastic syndrome (MDS) with low-percentage bone marrow blasts and predictive factors for tr...Objective To explore the outcome of cyclosporine A(CsA) combined with danazol with or without thalidomide regimen for myelodysplastic syndrome (MDS) with low-percentage bone marrow blasts and predictive factors for treatment response.展开更多
Objective To clarify the prevalence,clinical features and molecular characteristics of germline GATA2 mutations in pediatric primary myelodysplastic syndromes(MDS).Methods Next-generation sequencing technology was use...Objective To clarify the prevalence,clinical features and molecular characteristics of germline GATA2 mutations in pediatric primary myelodysplastic syndromes(MDS).Methods Next-generation sequencing technology was used to detect mutations in GATA2 and other myeloid malignancy genes in 129 children with primary MDS from Jan.2007 to Jan.2018.The relationship between genotypes and phenotypes was analyzed.Results Germline GATA2 mutations accounted for 8.5%(11/129)of all primary MDS cases,and 14.0%(11/50)of MDS with excess blasts(MDS-EB)and acute myeloid leukaemia with myelodysplasia-related changes(AMLMRC).Compared with GATA2 wild-type patients,GATA2 mutated patients were older at diagnosis[8(1-16)years old vs 6 years old(range:1 month old-18 years old),P=0.035]and higher risk of monosomy 7(72.7%vs 5.2%,P<0.001)and classified into MDS-EB and AML-MRC compared with refractory cytopenia of childhood(RCC)(63.6%vs 36.4%,P=0.111).The multivariate analysis showed SETBP1 mutation(P=0.041,OR=9.003,95%CI 1.098-73.787)and isolated monosomy 7(P=0.002,OR=24.835,95%CI 3.305-186.620)were significantly associated with germline mutated GATA2.Overall survival(OS)and outcomes of hematopoietic stem cell transplantation(HSCT)were not influenced by GATA2 mutational status.Conclusion Our data identify germline GATA2 mutations have a high prevalence in older pediatric patients with monosomy 7,and high risk of progression into advanced MDS subtypes.GATA2 mutation status does not affect OS in pediatric primary MDS.展开更多
Objective To quantitatively analyze the reticulin fiber intensity density (RFD) in patients with myelodysplastic syndrome (MDS) by using the computer-aided grid point method, and preliminarily explore its correlation ...Objective To quantitatively analyze the reticulin fiber intensity density (RFD) in patients with myelodysplastic syndrome (MDS) by using the computer-aided grid point method, and preliminarily explore its correlation with the prognosis of MDS patients.展开更多
文摘Therapeutic options for gastric variceal bleeding in the presence of extensive portal vein thrombosis associated with a myeloproliferative disorder are limited.We report a case of a young woman who presented with gastric variceal bleeding secondary to extensive splanchnic venous thrombosis due to a Janus kinase 2 mutation associated myeloproliferative disorder that was managed effectively with partial splenic embolization.
文摘This experiment was designed to study about the regulation and mechanisms of Buzhong Yiqi-Pill on bone marrow hema topoietic system.Bone marrow inhibition of mouse model was established to simulate blood deficiency syndrome.Semi-solid agar culture assay of hematopoietic progenitor cells(HPC)in vitro and flow cytometry were used.To study the effect of Buzhong Yiqi-Pill on bone marrow hematopoietic function of myelosuppressed mice,HPC survival,colony forming rate,bone marrow call cycle and cell apoptosis was detected.
文摘This study was to determine whether GM-CSF induced WT1 gene expression and to establish an association with markers of proliferation CD71+CD34+ using nPCR and flow cytometry respectively, in samples obtained from 5 newly diagnosed JMML patients. Overtime (day 0 to day 14) there was an insignificant difference in WT1 gene expression and CD71+CD34+ in JMML samples when compared to peripheral blood of normal volunteers (n = 3). Our study suggests that there is a correlation between WT1 gene expression and cellular proliferation and that GMCSF in vitro does not create a significant difference in JMML samples.
基金supported by grants from the Tianjin Health Science and Technology Project(No.TJWJ2022MS001)Clinical research project of Tianjin Society of Hematology and Regenerative Medicine(No.2022 TSHRM08004)+3 种基金Key Project of Tianjin Natural Science Foundation(No.20JCZDJC00410)CAMS Innovation Fund for Medical Sciences(No.2021-I2M-1-073)Haihe Laboratory of Cell Ecosystem Innovation Fund(No.22HHXBSS00034)National Natural Science Foundation of China(Nos.82070192,8230012348,and 82170217)
文摘To the Editor:Allogeneic hemopoietic stem cell transplantation(allo-HSCT)represents the only long-term survival treatment choice for patients with myelodysplastic syndromes(MDS)and MDS/myeloproliferative neoplasm(MPN).Unfortunately,post-hemopoietic stem cell transplantation(HSCT)relapse remains a cause of treatment failure and the results of salvage treatments are poor.Developing better conditioning regimens is urgently needed.Previous studies have shown synergistic antileukemic effects between decitabine(DEC)and idarubicin(IDA).[1]In an attempt to design a conditioning strategy with very low toxicity but considerable myelosuppressive activity and potential immune-enhancing effects for patients with high-risk MDS and MDS/MPN,we combined DEC and IDA with busulfan,cyclophosphamide,andudarabine for a modied myeloablative regimen in this prospective,multicenter cohort study(hereafter referred to as the“DEC/IDA study”).
基金This work wassupported by the National Natural Science Foundation of China (Grant No. 39970818).
文摘In the treatment of tumor patients introduction of multidrug resistance genes into hematopoietic cells has been reported as an approach for reducing myelotoxicity created by antitumor drugs. However, the nonspecific expression of the genes can also increase the chemoresistance of the tumor cells invaded into bone marrow, which influences seriously the effectiveness of chemotherapy. In this study, a new strategy is described for specific myeloprotection. The recombinant retroviral vector containing multidrug resistance 1 (MDR1) gene regulated by aminopeptidase N (APN) myeloid promoter was constructed and then introduced into myeloblastic cells KGla and tumor cell line BEL7402. The specific transcript of MDR1 was detected in KGla cells transduced with MDR1 gene and rhodamine 123 was effectively extruded by Pgp, the protein of MDR1 gene. The resistance elevated markedly by 10.6, 10.4, 11.2, 4.2 and 14.2 folds in MDR1 gene-transduced KGla cells to chemothera-peutic drugs such as cochicine, VP-16,
文摘In a recently published study in The New England Journal of Medicine,Liu and colleagues were able to demonstrate that-apart from the antileukemic effects previously reported for hypomethylating agents(HMAs)-treatment with these drugs can also lead to demethylation and subsequent upregulation of oncogenes resulting in shorter survival in patients with myelo-dysplastic syndrome(MDS).
文摘Objective To detect the expression level of growth differentiation factor 11(GDF11)in patients with myelodysplastic syndrome(MDS),and to evaluate the relationship between GDF11 level and erythropoiesis functions.Methods A total of 44 MDS patients(18 low-risk group patients and 26 high-risk group patients)in the Department of Hematology in Tianjin Medical
文摘Objective To assess the efficacy and toxicity of decitabine-based conditioning regimen in patients with myelodysplastic syndrome(MDS),acute myeloid leukemia secondary to MDS(MDS-AML)or chronic myelomonocytic leukemia(CMML).Methods From March 1,2013 to May 25,2015,22 patients who underwent allogenic hematopoietic stem cell transplantation(allo-HSCT)with decitabine-based conditioning regimen were analyzed retrospectively.Results①22 patients,14 males and 8 females with a median age of 42.5(24-56)years old,were diagnosed as MDS(n=14),CMML(n=4),MDS-AML(n=4).②15 patients were treated with the conditioning regimen of decitabine combined with busulfan,cyclophosphamide,fludarabine,and cytarabine,the other 7 cases were treated with decitabine,busulfan,fludarabine,and cytarabine.The dose of decitabine was 20 mg·m^-2·d^-1 for 5 days.Rabbit anti-human anti-thymocyte globulin(2.5 mg·kg^-1·d^-1 for 4 days)was involved in conditioning regimen in patients with unrelated donor or haploidentical transplantation.③Except 1 patient died of infection in 2 months after transplantation,the other patients were engrafted successfully.The median time of granulocyte engraftment was 13(12-18)days,and the median time of platelet engraftment was 16(13-81)days.④The incidence of acute graft versus host disease(aGVHD)was(41.3±10.6)%,and severe aGVHD(grade ofⅢ-Ⅳ)was(18.4±9.7)%.The incidence of chronic graft versus host disease(cGVHD)was(56.4±11.3)%,and extensive cGVHD was(36.4±12.1)%.⑤8 patients were suffered with cytomegalovirus(CMV)viremia.Among the 18 patients with definite infection,6 occurred during myelosuppression and 12 cases occurred after hematopoietic reconstruction.The 2-year and 3-year nonrelapse mortality was(13.9±7.4)%and(24.3±9.5)%,respectively.⑥The 2-year and 3-year overallsurvival(OS)was(77.3±8.9)%and(67.9±10.0)%,respectively.The 2-year and 3-year relapsefreesurvival(RFS)was(72.7±9.5)%and(63.6±10.3)%,respectively.Conclusion allo-HSCT withdecitabine-based conditioning regimen is feasible in thetreatment of MDS,MDS-AML or CMML.
文摘Objective To explore the outcome of cyclosporine A(CsA) combined with danazol with or without thalidomide regimen for myelodysplastic syndrome (MDS) with low-percentage bone marrow blasts and predictive factors for treatment response.
文摘Objective To clarify the prevalence,clinical features and molecular characteristics of germline GATA2 mutations in pediatric primary myelodysplastic syndromes(MDS).Methods Next-generation sequencing technology was used to detect mutations in GATA2 and other myeloid malignancy genes in 129 children with primary MDS from Jan.2007 to Jan.2018.The relationship between genotypes and phenotypes was analyzed.Results Germline GATA2 mutations accounted for 8.5%(11/129)of all primary MDS cases,and 14.0%(11/50)of MDS with excess blasts(MDS-EB)and acute myeloid leukaemia with myelodysplasia-related changes(AMLMRC).Compared with GATA2 wild-type patients,GATA2 mutated patients were older at diagnosis[8(1-16)years old vs 6 years old(range:1 month old-18 years old),P=0.035]and higher risk of monosomy 7(72.7%vs 5.2%,P<0.001)and classified into MDS-EB and AML-MRC compared with refractory cytopenia of childhood(RCC)(63.6%vs 36.4%,P=0.111).The multivariate analysis showed SETBP1 mutation(P=0.041,OR=9.003,95%CI 1.098-73.787)and isolated monosomy 7(P=0.002,OR=24.835,95%CI 3.305-186.620)were significantly associated with germline mutated GATA2.Overall survival(OS)and outcomes of hematopoietic stem cell transplantation(HSCT)were not influenced by GATA2 mutational status.Conclusion Our data identify germline GATA2 mutations have a high prevalence in older pediatric patients with monosomy 7,and high risk of progression into advanced MDS subtypes.GATA2 mutation status does not affect OS in pediatric primary MDS.
文摘Objective To quantitatively analyze the reticulin fiber intensity density (RFD) in patients with myelodysplastic syndrome (MDS) by using the computer-aided grid point method, and preliminarily explore its correlation with the prognosis of MDS patients.
