Objective To investigate the efficacy and safety of recombinant human interferon (rh-IFN) α-2b in treatment of pediatric immune thrombocytopenia (ITP) as a second-line therapy regimen. Methods From October 2015 to September 2017, a total of 95 ITP children who were not respond to first-line therapy regimen or glucocorticosteroid-dependent, and receiving rh-IFNα-2b treatment, were selected as study subjects after admitted to Department of Hematology and Oncology, Kunming Children′s Hospital. The average age of the children was 5.3 years. And 53 cases were male and 42 were female, and the gender composition ratio of male and female was 1.3∶1. Clinical characteristics of the children were collected retrospectively, and the curative effect and treatment-related adverse reactions were analyzed. The chi-square test was used to compare the total effective rate of children of different genders, age, and disease duration; the Bonferroni correction was used in pairwise comparisons of total effective rates of children with different disease dunration. The procedure of this study is accordance with the requirement of the revised World Medical Association Declaration of Helsinki in 2013. Informed consents were obtained from all participants′ guardians. Results ① Total effective rate of 95 ITP children treated by rh-IFNα-2b was 53.7% (51/95). And among 95 ITP children, 33 cases (34.7%) were continuously effective during the period of follow-up. And 18 cases (19.0%) were relapsed after rh-IFNα-2b treatment of short-term effective condition and responded to first-line treatment, and 2 cases (2.1%) were relapsed after rh-IFNα-2b treatment of short-term effective condition and did not respond to first-line treatment and rh-IFNα-2b. 34 cases (35.8%) were ineffective after treatment of rh-IFNα-2b. Two cases (2.1%) were discontinued after the first use of the drug due to high fever, 2 cases (2.1%) were discontinued by themselves and 4 cases (4.2%) were lost during the follow-up. ② There was no significant difference in the total effective rate among ITP children with different gender and age (P>0.05). The total effective rates of children with duration of disease less than or equal to 3 months, 3-12 months, and more than 12 months, were 45.4% (30/66), 75.0% (15/20) and 66.7% (6/9), respectively. And there was significant difference among them (χ2=6.282, P=0.043). However, there was no significant difference in pairwise comparison of the total effective rates of ITP children with different disease duration (P>0.017). ③ Among 95 cases of ITP children, 20 cases (21.1%) developed flu-like symptoms after the first dose, mainly including fever, headache, fatigue, and muscle pain. Among them, 4 patients (4.2%) had high fever (maximum temperature of 39 ℃), and 2 cases of them were withdrawn. 16 cases (16.8%) had mild fever (maximum temperature of 38 ℃). Except for 2 children who discontinued rh-IFNα-2b due to intolerable severe flu-like symptoms, symptoms of the
{"title":"Clinical analysis of recombinant human interferon α-2b in treatment of immune thrombocytopenia children who failed to first-line therapy","authors":"Chen Zhang, Na Li, Gangling Pu, Chunlian Fang, Yahui Liu, Chunguang Yang, Xin Tian","doi":"10.3760/CMA.J.ISSN.1673-419X.2019.02.008","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1673-419X.2019.02.008","url":null,"abstract":"Objective \u0000To investigate the efficacy and safety of recombinant human interferon (rh-IFN) α-2b in treatment of pediatric immune thrombocytopenia (ITP) as a second-line therapy regimen. \u0000 \u0000 \u0000Methods \u0000From October 2015 to September 2017, a total of 95 ITP children who were not respond to first-line therapy regimen or glucocorticosteroid-dependent, and receiving rh-IFNα-2b treatment, were selected as study subjects after admitted to Department of Hematology and Oncology, Kunming Children′s Hospital. The average age of the children was 5.3 years. And 53 cases were male and 42 were female, and the gender composition ratio of male and female was 1.3∶1. Clinical characteristics of the children were collected retrospectively, and the curative effect and treatment-related adverse reactions were analyzed. The chi-square test was used to compare the total effective rate of children of different genders, age, and disease duration; the Bonferroni correction was used in pairwise comparisons of total effective rates of children with different disease dunration. The procedure of this study is accordance with the requirement of the revised World Medical Association Declaration of Helsinki in 2013. Informed consents were obtained from all participants′ guardians. \u0000 \u0000 \u0000Results \u0000① Total effective rate of 95 ITP children treated by rh-IFNα-2b was 53.7% (51/95). And among 95 ITP children, 33 cases (34.7%) were continuously effective during the period of follow-up. And 18 cases (19.0%) were relapsed after rh-IFNα-2b treatment of short-term effective condition and responded to first-line treatment, and 2 cases (2.1%) were relapsed after rh-IFNα-2b treatment of short-term effective condition and did not respond to first-line treatment and rh-IFNα-2b. 34 cases (35.8%) were ineffective after treatment of rh-IFNα-2b. Two cases (2.1%) were discontinued after the first use of the drug due to high fever, 2 cases (2.1%) were discontinued by themselves and 4 cases (4.2%) were lost during the follow-up. ② There was no significant difference in the total effective rate among ITP children with different gender and age (P>0.05). The total effective rates of children with duration of disease less than or equal to 3 months, 3-12 months, and more than 12 months, were 45.4% (30/66), 75.0% (15/20) and 66.7% (6/9), respectively. And there was significant difference among them (χ2=6.282, P=0.043). However, there was no significant difference in pairwise comparison of the total effective rates of ITP children with different disease duration (P>0.017). ③ Among 95 cases of ITP children, 20 cases (21.1%) developed flu-like symptoms after the first dose, mainly including fever, headache, fatigue, and muscle pain. Among them, 4 patients (4.2%) had high fever (maximum temperature of 39 ℃), and 2 cases of them were withdrawn. 16 cases (16.8%) had mild fever (maximum temperature of 38 ℃). Except for 2 children who discontinued rh-IFNα-2b due to intolerable severe flu-like symptoms, symptoms of the","PeriodicalId":13774,"journal":{"name":"International Journal of Blood Transfusion and Hematology","volume":"42 1","pages":"134-138"},"PeriodicalIF":0.0,"publicationDate":"2019-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43823533","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-03-20DOI: 10.3760/CMA.J.ISSN.1673-419X.2019.02.010
W. Fu
Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL) is a new high-risk subtype of acute lymphoblastic leukemia (ALL) proposed in recent years. It is characterized by a gene-expression profile similar to Ph-positive ALL, involving abnormal activation of cytokine receptor and tyrosine kinase, and commonly harbor genetic alterations targeting lymphoid transcription factors, and associated with a poor prognosis. Clinical studies showed that targeted inhibitors of related signaling pathways can significantly improve the prognosis in specific patients. The research progress of Ph-like ALL is summarized in this review. Key words: Philadelphia chromosome; Precursor cell lymphoblastic leukemia-lymphoma; Abelson murine leukemia virus; Prognosis; CRLF2 gene
{"title":"Research progress of Ph-like acute lymphoblastic leukemia","authors":"W. Fu","doi":"10.3760/CMA.J.ISSN.1673-419X.2019.02.010","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1673-419X.2019.02.010","url":null,"abstract":"Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL) is a new high-risk subtype of acute lymphoblastic leukemia (ALL) proposed in recent years. It is characterized by a gene-expression profile similar to Ph-positive ALL, involving abnormal activation of cytokine receptor and tyrosine kinase, and commonly harbor genetic alterations targeting lymphoid transcription factors, and associated with a poor prognosis. Clinical studies showed that targeted inhibitors of related signaling pathways can significantly improve the prognosis in specific patients. The research progress of Ph-like ALL is summarized in this review. \u0000 \u0000 \u0000Key words: \u0000Philadelphia chromosome; Precursor cell lymphoblastic leukemia-lymphoma; Abelson murine leukemia virus; Prognosis; CRLF2 gene","PeriodicalId":13774,"journal":{"name":"International Journal of Blood Transfusion and Hematology","volume":"42 1","pages":"143-148"},"PeriodicalIF":0.0,"publicationDate":"2019-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45992601","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-01-20DOI: 10.3760/CMA.J.ISSN.1673-419X.2019.01.009
Y. Liao, Mengyao Wang
The treatment of multiple myeloma (MM) has developed rapidly in the past decade, due to the notable effect of protease inhibitors (PI) and immunomodulatory drugs (IMID). However, MM remains incurable in the vast majority of cases. Recently, a large number of studies have found that monoclonal antibodies, such as a targeted approach can significantly increase remission rate or survival for patients with MM, including relapsed/refractory multiple myeloma (RRMM) and newly diagnosed multiple myeloma (NDMM). This review focuses on the recent developments in monoclonal antibody-based therapy for MM in terms of targets, efficacy and safety. Key words: Multiple myeloma; Antibodies, monoclonal; Antigens; Molecular targeted therapy; Treatment outcome; Clinical protocols; Drug-related side effects and adverse reactions
{"title":"Research progress of monoclonal antibodies in treatment of multiple myeloma","authors":"Y. Liao, Mengyao Wang","doi":"10.3760/CMA.J.ISSN.1673-419X.2019.01.009","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1673-419X.2019.01.009","url":null,"abstract":"The treatment of multiple myeloma (MM) has developed rapidly in the past decade, due to the notable effect of protease inhibitors (PI) and immunomodulatory drugs (IMID). However, MM remains incurable in the vast majority of cases. Recently, a large number of studies have found that monoclonal antibodies, such as a targeted approach can significantly increase remission rate or survival for patients with MM, including relapsed/refractory multiple myeloma (RRMM) and newly diagnosed multiple myeloma (NDMM). This review focuses on the recent developments in monoclonal antibody-based therapy for MM in terms of targets, efficacy and safety. \u0000 \u0000 \u0000Key words: \u0000Multiple myeloma; Antibodies, monoclonal; Antigens; Molecular targeted therapy; Treatment outcome; Clinical protocols; Drug-related side effects and adverse reactions","PeriodicalId":13774,"journal":{"name":"International Journal of Blood Transfusion and Hematology","volume":"42 1","pages":"53-60"},"PeriodicalIF":0.0,"publicationDate":"2019-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49610487","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-01-20DOI: 10.3760/CMA.J.ISSN.1673-419X.2019.01.008
Hongyan Yu, Gui-Bo Yang, A. Tang
Thrombocytopenia is a common disease in the clinic. In severe cases, it can be life-threatening. Thrombopoietin receptor agonists (TPO-RA) can effectively increase platelet counts in patients with thrombocytopenia and reduce blood transfusion dependence. As the second-generation TPO-RA, eltrombopag (EP) has been approved for thrombocytopenia caused by chronic immune thrombocytopenic purpura (cITP), severe aplastic anemia(SAA), and infection of chronic hepatitis C (HCV). This article reviews the progress of the application of EP in thrombocytopenia. Key words: Receptors, thrombopoietin; Thrombocytopenia; Hepatitis C; Anemia, aplastic; Eltrombopag
{"title":"Application progress of eltrombopag in thrombocytopenia","authors":"Hongyan Yu, Gui-Bo Yang, A. Tang","doi":"10.3760/CMA.J.ISSN.1673-419X.2019.01.008","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1673-419X.2019.01.008","url":null,"abstract":"Thrombocytopenia is a common disease in the clinic. In severe cases, it can be life-threatening. Thrombopoietin receptor agonists (TPO-RA) can effectively increase platelet counts in patients with thrombocytopenia and reduce blood transfusion dependence. As the second-generation TPO-RA, eltrombopag (EP) has been approved for thrombocytopenia caused by chronic immune thrombocytopenic purpura (cITP), severe aplastic anemia(SAA), and infection of chronic hepatitis C (HCV). This article reviews the progress of the application of EP in thrombocytopenia. \u0000 \u0000 \u0000Key words: \u0000Receptors, thrombopoietin; Thrombocytopenia; Hepatitis C; Anemia, aplastic; Eltrombopag","PeriodicalId":13774,"journal":{"name":"International Journal of Blood Transfusion and Hematology","volume":"42 1","pages":"46-52"},"PeriodicalIF":0.0,"publicationDate":"2019-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45314790","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-01-20DOI: 10.3760/CMA.J.ISSN.1673-419X.2019.01.013
P. Dong
Myeloid-derived suppressor cells (MDSC) are a heterogeneous group of immature myeloid cells that suppress the innate and adaptive immune system via different mechanisms and accumulate under pathological conditions, such as inflammation, cancer and autoimmune disease. It plays a role in the progression of cancer and immune evasion. Increased number of MDSC has been reported in hematologic malignant diseases, such as leukemia and lymphoma and so on, and is associated with a poor clinical outcome. This review aims to review the advance in pathophysiological mechanism of MDSC in hematologic malignancies and the role of MDSC in allogeneic hematopoietic stem cell transplantation (allo-HSCT). Key words: Myeloid-derived suppressor cells; Hematologic malignancy; Leukemia; Lymphoma; Immunotherapy
{"title":"Myeloid-derived suppressor cells and hematologic malignant diseases","authors":"P. Dong","doi":"10.3760/CMA.J.ISSN.1673-419X.2019.01.013","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1673-419X.2019.01.013","url":null,"abstract":"Myeloid-derived suppressor cells (MDSC) are a heterogeneous group of immature myeloid cells that suppress the innate and adaptive immune system via different mechanisms and accumulate under pathological conditions, such as inflammation, cancer and autoimmune disease. It plays a role in the progression of cancer and immune evasion. Increased number of MDSC has been reported in hematologic malignant diseases, such as leukemia and lymphoma and so on, and is associated with a poor clinical outcome. This review aims to review the advance in pathophysiological mechanism of MDSC in hematologic malignancies and the role of MDSC in allogeneic hematopoietic stem cell transplantation (allo-HSCT). \u0000 \u0000 \u0000Key words: \u0000Myeloid-derived suppressor cells; Hematologic malignancy; Leukemia; Lymphoma; Immunotherapy","PeriodicalId":13774,"journal":{"name":"International Journal of Blood Transfusion and Hematology","volume":"42 1","pages":"79-84"},"PeriodicalIF":0.0,"publicationDate":"2019-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43055462","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-01-20DOI: 10.3760/CMA.J.ISSN.1673-419X.2019.01.002
Su Liu, Shaofen Lin, Qihui Chen, H. Xue, Hong-gui Xu, Shao-liang Huang, Chun Chen
Objective To evaluate efficacy, complications and influencing factors of children with aplastic anemia(AA)who were receiving rabbit anti-thymocyte globulin (rATG) combined with cyclosporine for immunosuppressive therapy (IST), and to explore optimal therapy for different clinical types of AA according to severity. Methods From 1st January 2006 to 1st February 2017, a total of 128 children who were diagnosed as AA by peripheral blood routine and bone marrow morphology and/or bone marrow pathology, and accepted rATG combined with cyclosporine in Department of Pediatric, Sun Yat-sen Memorial Hospital, Sun Yat-sen University. According to the clinical classification of the disease, the subjects were divided into non-severe aplastic anemia (NSAA) group (n=22), severe aplastic anemia (SAA) group (n=63), and very severe aplastic anemia (VSAA) group (n=43). The clinical data of the patients were retrospectively analyzed. The efficacy of the patients at 3, 6, 9 and 12 months after treatment and at the end of follow-up among 3 groups were evaluated by chi-square test. The treatment-related adverse reactions and prognosis were observed. Univariate analysis of different influencing factors, such as age, absolute value of neutrophils, platelet count, and reticulocyte were performed using the chi-square test. Statistically significant factors in the univariate analysis were included in the Cox proportional hazard regression model for multivariate analysis. Survival analysis was performed using the Kaplan-Meier method. The procedure of this study was accordance with the requirement of the revised World Medical Association Declaration of Helsinki in 2013. Informed consent was obtained from all guardians of participants. Results ① Of the 128 patients, 121 cases were evaluable, 5 cases were lost to follow-up, and 2 cases were early death. The complete remission (CR) rates at 3, 6, 9, and 12 months after treatment and at the end of follow-up were 5.0% (6/121), 14.9% (18/121), 16.5%(20/121), 28.9% (35/121) and 51.2% (62/121), respectively. The overall response rates were 33.9% (41/121), 38.9% (47/121), 49.6% (60/121), 62.8% (76/121) and 65.3% (79/121) , respectively. The response rates at 3 and 6 months after treatment in the NSAA group were 63.6% (14/22) and 68.2% (15/22), which were significantly higher than those of 30.5% (18/59) and 39.0% (23/59) in SAA group, 22.5% (9/40) and 22.5% (9/40) in VSAA group, the former were statistically different from the latter two (χ2=11.416, 13.297, P=0.001, 0.05). ④ The lymphocyte count 0.05). Conclusions The treatment of rATG combined with cyclosporine in children with SAA and VSAA has a higher response rate and a lower incidence of serious complications, which can be used as first-line treatment in the absence of appropriate donors. The lymphocyte count<1.5×109/L, platelet count≥20×109/L, and decreased CD3+ CD4+ cell ratio might be predictors of good response to rATG combined with cyclosporine in the treatm
{"title":"Clinical analysis on efficacy of rabbit anti-thymocyte globulin combined with cyclosporine in treatment of 128 children with aplastic anemia","authors":"Su Liu, Shaofen Lin, Qihui Chen, H. Xue, Hong-gui Xu, Shao-liang Huang, Chun Chen","doi":"10.3760/CMA.J.ISSN.1673-419X.2019.01.002","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1673-419X.2019.01.002","url":null,"abstract":"Objective \u0000To evaluate efficacy, complications and influencing factors of children with aplastic anemia(AA)who were receiving rabbit anti-thymocyte globulin (rATG) combined with cyclosporine for immunosuppressive therapy (IST), and to explore optimal therapy for different clinical types of AA according to severity. \u0000 \u0000 \u0000Methods \u0000From 1st January 2006 to 1st February 2017, a total of 128 children who were diagnosed as AA by peripheral blood routine and bone marrow morphology and/or bone marrow pathology, and accepted rATG combined with cyclosporine in Department of Pediatric, Sun Yat-sen Memorial Hospital, Sun Yat-sen University. According to the clinical classification of the disease, the subjects were divided into non-severe aplastic anemia (NSAA) group (n=22), severe aplastic anemia (SAA) group (n=63), and very severe aplastic anemia (VSAA) group (n=43). The clinical data of the patients were retrospectively analyzed. The efficacy of the patients at 3, 6, 9 and 12 months after treatment and at the end of follow-up among 3 groups were evaluated by chi-square test. The treatment-related adverse reactions and prognosis were observed. Univariate analysis of different influencing factors, such as age, absolute value of neutrophils, platelet count, and reticulocyte were performed using the chi-square test. Statistically significant factors in the univariate analysis were included in the Cox proportional hazard regression model for multivariate analysis. Survival analysis was performed using the Kaplan-Meier method. The procedure of this study was accordance with the requirement of the revised World Medical Association Declaration of Helsinki in 2013. Informed consent was obtained from all guardians of participants. \u0000 \u0000 \u0000Results \u0000① Of the 128 patients, 121 cases were evaluable, 5 cases were lost to follow-up, and 2 cases were early death. The complete remission (CR) rates at 3, 6, 9, and 12 months after treatment and at the end of follow-up were 5.0% (6/121), 14.9% (18/121), 16.5%(20/121), 28.9% (35/121) and 51.2% (62/121), respectively. The overall response rates were 33.9% (41/121), 38.9% (47/121), 49.6% (60/121), 62.8% (76/121) and 65.3% (79/121) , respectively. The response rates at 3 and 6 months after treatment in the NSAA group were 63.6% (14/22) and 68.2% (15/22), which were significantly higher than those of 30.5% (18/59) and 39.0% (23/59) in SAA group, 22.5% (9/40) and 22.5% (9/40) in VSAA group, the former were statistically different from the latter two (χ2=11.416, 13.297, P=0.001, 0.05). ④ The lymphocyte count 0.05). \u0000 \u0000 \u0000Conclusions \u0000The treatment of rATG combined with cyclosporine in children with SAA and VSAA has a higher response rate and a lower incidence of serious complications, which can be used as first-line treatment in the absence of appropriate donors. The lymphocyte count<1.5×109/L, platelet count≥20×109/L, and decreased CD3+ CD4+ cell ratio might be predictors of good response to rATG combined with cyclosporine in the treatm","PeriodicalId":13774,"journal":{"name":"International Journal of Blood Transfusion and Hematology","volume":"42 1","pages":"9-17"},"PeriodicalIF":0.0,"publicationDate":"2019-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41990153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-01-20DOI: 10.3760/CMA.J.ISSN.1673-419X.2019.01.012
Lang Peng
Studies show that DNA methylation play an important role during the transformation of hematopoitic diseases. Decitabine is a kind of demethylation drug that can inhibit DNA methyltransferase. Currently, decitabine is mainly used to treat hematological diseases, such as myelodysplastic syndrome (MDS), acute myeloid leukemia (AML), chronic myelomonocytic leukemia (CMML), etc., due to its unique mechanisms and better clinical effectiveness. In recent years, decitabine is also used for steroid-resistant/refractory primary immune thrombocytopenia (ITP) patients, which can improve platelet count, reduce bleeding risk and improve patients′ prognosis. In order to explore the clinical application of decitabine, this article summarizes the research progress on decitabine for treatment of hematopoietic disease. Key words: Myelodysplastic syndromes; Hematologic diseases; Leukemia, myeloid; Purpura, thrombocytopenic, idiopathic; Decitabine
{"title":"Research progress of decitabine in treatment of hematologic diseases","authors":"Lang Peng","doi":"10.3760/CMA.J.ISSN.1673-419X.2019.01.012","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1673-419X.2019.01.012","url":null,"abstract":"Studies show that DNA methylation play an important role during the transformation of hematopoitic diseases. Decitabine is a kind of demethylation drug that can inhibit DNA methyltransferase. Currently, decitabine is mainly used to treat hematological diseases, such as myelodysplastic syndrome (MDS), acute myeloid leukemia (AML), chronic myelomonocytic leukemia (CMML), etc., due to its unique mechanisms and better clinical effectiveness. In recent years, decitabine is also used for steroid-resistant/refractory primary immune thrombocytopenia (ITP) patients, which can improve platelet count, reduce bleeding risk and improve patients′ prognosis. In order to explore the clinical application of decitabine, this article summarizes the research progress on decitabine for treatment of hematopoietic disease. \u0000 \u0000 \u0000Key words: \u0000Myelodysplastic syndromes; Hematologic diseases; Leukemia, myeloid; Purpura, thrombocytopenic, idiopathic; Decitabine","PeriodicalId":13774,"journal":{"name":"International Journal of Blood Transfusion and Hematology","volume":"42 1","pages":"72-78"},"PeriodicalIF":0.0,"publicationDate":"2019-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46661302","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-01-20DOI: 10.3760/CMA.J.ISSN.1673-419X.2019.01.014
Yingying Chen, Lihong Wang
Iron is the most basic element involved in oxygen transport, cell respiration, DNA replication, and other disorders of iron metabolism associated with a variety of diseases, including anemia (iron deficiency anemia, chronic anemia) and iron overload (hemochromatosis). Hepcidin is a small molecule peptide hormone synthesized by the liver, which plays a negative role in regulating iron metabolism and is particularly important for maintaining iron balance. Hepcidin is encoded by Hamp gene, and its expression is regulated by multiple signaling pathways such as BMP and STAT. Signal molecules such as interleukin (IL)-6 and transmembrane serine protease (TMPRSS)6 also play important roles in the regulation of hepcidin. This article reviews the molecular structure and role of hepcidin, as well as the current research progress on regulatory drugs targeting hepcidin, Hamp gene and its related signaling pathways. Key words: Iron; Iron metabolism disorders; Hemochromatosis; Interleukin-6; Hepcidin; Hamp gene
{"title":"Research progress on hepcidin and its related regulatory drugs","authors":"Yingying Chen, Lihong Wang","doi":"10.3760/CMA.J.ISSN.1673-419X.2019.01.014","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1673-419X.2019.01.014","url":null,"abstract":"Iron is the most basic element involved in oxygen transport, cell respiration, DNA replication, and other disorders of iron metabolism associated with a variety of diseases, including anemia (iron deficiency anemia, chronic anemia) and iron overload (hemochromatosis). Hepcidin is a small molecule peptide hormone synthesized by the liver, which plays a negative role in regulating iron metabolism and is particularly important for maintaining iron balance. Hepcidin is encoded by Hamp gene, and its expression is regulated by multiple signaling pathways such as BMP and STAT. Signal molecules such as interleukin (IL)-6 and transmembrane serine protease (TMPRSS)6 also play important roles in the regulation of hepcidin. This article reviews the molecular structure and role of hepcidin, as well as the current research progress on regulatory drugs targeting hepcidin, Hamp gene and its related signaling pathways. \u0000 \u0000 \u0000Key words: \u0000Iron; Iron metabolism disorders; Hemochromatosis; Interleukin-6; Hepcidin; Hamp gene","PeriodicalId":13774,"journal":{"name":"International Journal of Blood Transfusion and Hematology","volume":"42 1","pages":"85-89"},"PeriodicalIF":0.0,"publicationDate":"2019-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46712833","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-01-20DOI: 10.3760/CMA.J.ISSN.1673-419X.2019.01.006
Yanbin Xiao, Shu-chuan Liu
Immune thrombocytopenia (ITP) is an acquired autoimmune hemorrhagic disease involving increased platelet destruction and insufficient platelet production, leading to thrombocytopenia, with or without clinical manifestations of bleeding. Although thrombocytopenia may cause coagulation dysfunction and hemorrhagic complications, the bleeding severity is not certainly proportional to the number of platelets. In some cases, there is no significant bleeding symptom even the platelet count is extremely low, which suggests there are additional compensatory mechanisms to control or ameliorate the bleeding complications in ITP patients. The present article aims to review recent investigations on the compensatory mechanisms of coagulation function in the patients with ITP. Key words: Thrombocytopenia; Microsomes; Phosphatidylserines; Thromboplastin; Platelet membrane glycoproteins; Thrombin; Interleukin-6
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Pub Date : 2019-01-20DOI: 10.3760/CMA.J.ISSN.1673-419X.2019.01.004
Xing Li, Zongxiang Sun, Tao Yin, Z. Song, Wenzhen Yu
Objective To investigate the current status of clinical blood transfusion in Yantai City from 2015 to 2016, analyze the weak links of clinical blood transfusion, and explore ways to strengthen clinical blood transfusion management. Methods Form 2015 to 2016, a total of 62 hospitals that signed blood supply agreements with the Yantai Central Blood Central Station were selected as research objects. According to the levels and charactoristics of the 62 hospitals, they were divided into tertiary general and public hospital group (n=12), secondary general and public hospital group (n=21), maternal and child health hospital group (n=5), first-class general and public hospital group (n=12), and private hospital group (n=12). In this study, primary hospitals included maternal and child health hospitals, first-class general and public hospitals and private hospitals. According to the Hospital Blood Transfusion Quality Evaluation and Assessment Form in Yantai City, on-site assessment of the infrastructure related to clinical blood transfusion in 62 hospitals was conducted. The questionnaires were used to investigate the implementaton of autologous blood transfusion in the 62 hospitals. A total of 510 clinical blood transfusion application forms and 270 clinical blood transfusion records were sampled randomly from 62 hospitals in January to June 2016, and the qualification rates of clinical blood transfusion application forms and clinical transfusion records were investigated. The chi-square test or the continuous correction chi-square test was used to compare the differences among hospitals of different groups or levels, which included proportions of hospitals evaluated by indicators of infrastructure related to clinical blood transfusion, implementaton rates of autologous blood transfusion, qualification rates of clinical blood transfusion application forms, and the composition ratio of clinical blood transfusion records of different grades. Results ① Among the 62 hospitals of this study, the proportion of hospitals that built clinical blood transfusion-related information management systems was 19.4% (12/62), proportion of hospitals that connected information with blood stations by internet was 8.1% (5/62), proportion of hospitals with separate blood transfusion department was 50.0% (31/62), proportion of hospitals participating in inter-room quality evaluation was 53.2% (33/62), proportion of hospitals that regularly organized clinical blood transfusion-related staffs to train was 77.4% (48/62), proportion of hospitals that took effective measures to evaluate reasonable clinical blood was 40.3% (25/62). The above 6 indicators of the secondary and tertiary general and public hospitals were significantly higher than those of the primary hospitals, and the differences were statistically significant (χ2=8.832, P=0.003; χ2=4.779, P=0.029; χ2=34.802, P<0.001; χ2=34.030, P<0.001; χ2=4.415, P=0.036; χ2=15.936, P<0.001). ② The total implementaton ra
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