Background: Because knowledge of blood donor motivation is crucial in guiding recruitment and retention efforts, the present study aimed at developing and validating a new scale as a multidimensional measure of blood donation motivation from the perspective of the self- determination theory.
Methods: This study was conducted in three phases from September 2022 to May 2023. The first phase involved developing a draft scale based on a literature review. In the second phase, face and content validity were performed. The third phase used a cross-sectional design to assess the construct validity and internal consistency of the initial scale following administration to blood donors with a history of at least one previous whole blood donation who visited the largest blood transfusion center in Iran. Of the 420 subjects who were recruited using a mixed sampling method, 343 who fully completed the initial version of the scale were subjected to an construct validity assessment using exploratory factor analysis with Equamax rotation and internal consistency using McDonald's omega coefficient.
Main results: The initial version of the scale consisted of 30 survey items with both a content validity ratio and a content validity index of 0.99. Exploratory factor analysis identified 24 items; grouped in six regulation factors (non-regulation, external regulation, introjected regulation, identified regulation, integrated regulation, and intrinsic regulation). The factors demonstrated adequate internal consistency with ω values ranging from 0.60 to 0.79.
Conclusion: The present study provides psychometric support for the newly developed questionnaire to evaluate donation motivation among blood donors in Iran or in other countries with similar language, and religious and cultural values.
{"title":"Development and validation of the self-regulation of blood donation scale for blood donors.","authors":"Fahimeh Ranjbar Kermani, Sedigheh Amini Kafi-Abad, Mahtab Maghsudlu, Kamran Mousavi Hosseini, Fatemeh Mohammadali, Atefeh MohammadJafari","doi":"10.1016/j.htct.2024.09.2482","DOIUrl":"https://doi.org/10.1016/j.htct.2024.09.2482","url":null,"abstract":"<p><strong>Background: </strong>Because knowledge of blood donor motivation is crucial in guiding recruitment and retention efforts, the present study aimed at developing and validating a new scale as a multidimensional measure of blood donation motivation from the perspective of the self- determination theory.</p><p><strong>Methods: </strong>This study was conducted in three phases from September 2022 to May 2023. The first phase involved developing a draft scale based on a literature review. In the second phase, face and content validity were performed. The third phase used a cross-sectional design to assess the construct validity and internal consistency of the initial scale following administration to blood donors with a history of at least one previous whole blood donation who visited the largest blood transfusion center in Iran. Of the 420 subjects who were recruited using a mixed sampling method, 343 who fully completed the initial version of the scale were subjected to an construct validity assessment using exploratory factor analysis with Equamax rotation and internal consistency using McDonald's omega coefficient.</p><p><strong>Main results: </strong>The initial version of the scale consisted of 30 survey items with both a content validity ratio and a content validity index of 0.99. Exploratory factor analysis identified 24 items; grouped in six regulation factors (non-regulation, external regulation, introjected regulation, identified regulation, integrated regulation, and intrinsic regulation). The factors demonstrated adequate internal consistency with ω values ranging from 0.60 to 0.79.</p><p><strong>Conclusion: </strong>The present study provides psychometric support for the newly developed questionnaire to evaluate donation motivation among blood donors in Iran or in other countries with similar language, and religious and cultural values.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142635094","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 : 2024-11-07DOI: 10.1016/j.htct.2024.06.012
Cilomar Martins de Oliveira Filho, Ibidunni Bode-Sojobi, Barbara D Lam, Stephanie Conrad, Jonathan Berry, Brian J Carney
{"title":"Assessing treatment response in thrombotic thrombocytopenic purpura: Beyond the platelet count.","authors":"Cilomar Martins de Oliveira Filho, Ibidunni Bode-Sojobi, Barbara D Lam, Stephanie Conrad, Jonathan Berry, Brian J Carney","doi":"10.1016/j.htct.2024.06.012","DOIUrl":"https://doi.org/10.1016/j.htct.2024.06.012","url":null,"abstract":"","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142690173","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}
Background: In transfusion-related iron overload, macrophage/reticuloendothelial cells are the first site of haem-derived iron accumulation. The prevention of haem-induced cytotoxicity in macrophages may represent a target for iron overload treatment. Deferasirox, an oral iron chelator, has been used to treat transfusion-related iron overload however, low adherence to the therapy is an issue. Statins, which are widely used for the prevention of atherosclerotic cardiovascular diseases, also have anti-oxidative and anti-inflammatory effects independent of their lipid lowering ones. Whether statins can suppress hemin-induced cytotoxicity and enhance the cytoprotective effects of deferasirox are important considerations to improve transfusion-related iron overload treatment. This study also evaluated the effects of eltrombopag, a thrombopoietin receptor agonist.
Materials and methods: Human monocytic THP-1 cells were pretreated with statins, deferasirox, and/or eltrombopag, followed by treatment with hemin. Cell viability, reactive oxygen species generation, and the intracellular labile iron pool were measured using flow cytometry.
Results: Fluvastatin and another four statins suppressed hemin-induced cell death, reactive oxygen species generation, and increases in the labile iron pool. Moreover, fluvastatin enhanced the suppressive effect of deferasirox on hemin-induced cell death. The effects of eltrombopag were similar to those of the statins.
Conclusion: The safety of statins is well established. When used in combination with fluvastatin or other statins, the suppressive effects of deferasirox on hemin-induced cytotoxicity in THP-1 cells were amplified. Further research is necessary to see whether statins will act in the same way in vivo or in human primary monocytes/macrophages.
{"title":"Fluvastatin suppresses hemin-induced cell death, reactive oxygen species generation, and elevated labile iron pool.","authors":"Shion Imoto, Katsuyasu Saigo, Mari Kono, Ayako Ohbuchi, Tohru Sawamura, Yuji Mizokoshi, Takashi Suzuki","doi":"10.1016/j.htct.2024.09.2480","DOIUrl":"https://doi.org/10.1016/j.htct.2024.09.2480","url":null,"abstract":"<p><strong>Background: </strong>In transfusion-related iron overload, macrophage/reticuloendothelial cells are the first site of haem-derived iron accumulation. The prevention of haem-induced cytotoxicity in macrophages may represent a target for iron overload treatment. Deferasirox, an oral iron chelator, has been used to treat transfusion-related iron overload however, low adherence to the therapy is an issue. Statins, which are widely used for the prevention of atherosclerotic cardiovascular diseases, also have anti-oxidative and anti-inflammatory effects independent of their lipid lowering ones. Whether statins can suppress hemin-induced cytotoxicity and enhance the cytoprotective effects of deferasirox are important considerations to improve transfusion-related iron overload treatment. This study also evaluated the effects of eltrombopag, a thrombopoietin receptor agonist.</p><p><strong>Materials and methods: </strong>Human monocytic THP-1 cells were pretreated with statins, deferasirox, and/or eltrombopag, followed by treatment with hemin. Cell viability, reactive oxygen species generation, and the intracellular labile iron pool were measured using flow cytometry.</p><p><strong>Results: </strong>Fluvastatin and another four statins suppressed hemin-induced cell death, reactive oxygen species generation, and increases in the labile iron pool. Moreover, fluvastatin enhanced the suppressive effect of deferasirox on hemin-induced cell death. The effects of eltrombopag were similar to those of the statins.</p><p><strong>Conclusion: </strong>The safety of statins is well established. When used in combination with fluvastatin or other statins, the suppressive effects of deferasirox on hemin-induced cytotoxicity in THP-1 cells were amplified. Further research is necessary to see whether statins will act in the same way in vivo or in human primary monocytes/macrophages.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142635097","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 : 2024-10-18DOI: 10.1016/j.htct.2024.09.2479
Rufeng Xie, Yiming Yang, Xueyu Jiang, Li Gao, Juan Sun, Jie Yang
Background: It is known that the rapid clearance of cold-stored platelets is attributed to various storage lesions, including an abnormal increase in reactive oxygen species when platelets are exposed to cold temperatures. As an antioxidant, N-acetylcysteine exhibits some significant effects on scavenging various reactive oxygen species and inhibiting cell damage and apoptosis.
Aims: This study aimed to investigate the effects of N-acetylcysteine on reducing reactive oxygen species production and protecting cold-stored platelets from phagocytosis and clearance, and to determine the optimal concentration of N-acetylcysteine.
Methods: Platelet concentrates were divided into three groups: room-temperature-stored platelets, cold-stored platelets, and cold-stored platelets with the addition of different concentrations of N-acetylcysteine. After five days of storage, reactive oxygen species production, lipid peroxidation levels, activation marker expressions, GPIb/ɑ desialylation with exposure of glycan residues and other quality parameters of platelets were measured and compared between the groups. Phagocytosis of platelets was detected by phorbol 12-myristate 13-acetate-activated THP-1 or Hep G2 cells. Moreover, the recovery of infused platelets was measured in severe combined immunodeficient mice at different timepoints.
Results: After 5 days of storage, cytoplasmic reactive oxygen species significantly increased in chilled compared to non-chilled platelets; they were notably reduced with the addition of N-acetylcysteine, particularly at a concentration of 5 mM. Compared with chilled platelets, the P-selectin and phosphatidylserine expressions, as well as exposure of GPIb/ɑ glycan residues, were significantly reduced with 5 mM of N-acetylcysteine. Phagocytosis of platelets by THP-1 or Hep G2 cells was significantly lower in 5 mM of N-acetylcysteine compared to cold-stored platelets without N-acetylcysteine.
Conclusions: This study demonstrated correlations between reactive oxygen species production and their pro-oxidant effects on platelet clearance after cold storage. The addition of N-acetylcysteine at an appropriate concentration do not only protects chilled platelets from storage lesions caused by reactive oxygen species overproduction but also prevents platelet phagocytosis in vitro and clearance in vivo, thereby extending circulating time.
背景:众所周知,冷藏血小板的快速清除是由于各种贮存病变造成的,包括血小板暴露于低温时活性氧的异常增加。目的:本研究旨在探讨 N-乙酰半胱氨酸对减少活性氧生成、保护冷藏血小板不被吞噬和清除的作用,并确定 N-乙酰半胱氨酸的最佳浓度:将浓缩血小板分为三组:室温储存血小板、低温储存血小板和添加不同浓度 N-乙酰半胱氨酸的低温储存血小板。储存五天后,测量并比较了各组血小板的活性氧生成、脂质过氧化水平、活化标志物表达、GPIb/ɑ去酰化与糖残基暴露以及其他质量参数。通过磷酸-12-肉豆蔻酸-13-乙酸酯激活的 THP-1 或 Hep G2 细胞检测血小板的吞噬作用。此外,还在不同时间点测定了严重合并免疫缺陷小鼠输注血小板的恢复情况:结果:储存 5 天后,冷藏血小板与非冷藏血小板相比,细胞质中的活性氧明显增加;加入 N-乙酰半胱氨酸后,活性氧明显减少,尤其是在浓度为 5 mM 时。与冷藏血小板相比,P-选择素和磷脂酰丝氨酸的表达以及 GPIb/ɑ 聚糖残基的暴露在 5 mM N-乙酰半胱氨酸的作用下明显减少。与不含 N-乙酰半胱氨酸的冷藏血小板相比,5 mM N-乙酰半胱氨酸可明显降低 THP-1 或 Hep G2 细胞对血小板的吞噬作用:本研究证明了活性氧的产生与它们对冷藏后血小板清除的促氧化作用之间的相关性。添加适当浓度的 N-乙酰半胱氨酸不仅能保护冷藏血小板免受活性氧生成过多导致的储存损伤,还能防止血小板在体外被吞噬和在体内被清除,从而延长循环时间。
{"title":"The effect of modulating platelet reactive oxygen species by the addition of antioxidants to prevent clearance of cold-stored platelets.","authors":"Rufeng Xie, Yiming Yang, Xueyu Jiang, Li Gao, Juan Sun, Jie Yang","doi":"10.1016/j.htct.2024.09.2479","DOIUrl":"https://doi.org/10.1016/j.htct.2024.09.2479","url":null,"abstract":"<p><strong>Background: </strong>It is known that the rapid clearance of cold-stored platelets is attributed to various storage lesions, including an abnormal increase in reactive oxygen species when platelets are exposed to cold temperatures. As an antioxidant, N-acetylcysteine exhibits some significant effects on scavenging various reactive oxygen species and inhibiting cell damage and apoptosis.</p><p><strong>Aims: </strong>This study aimed to investigate the effects of N-acetylcysteine on reducing reactive oxygen species production and protecting cold-stored platelets from phagocytosis and clearance, and to determine the optimal concentration of N-acetylcysteine.</p><p><strong>Methods: </strong>Platelet concentrates were divided into three groups: room-temperature-stored platelets, cold-stored platelets, and cold-stored platelets with the addition of different concentrations of N-acetylcysteine. After five days of storage, reactive oxygen species production, lipid peroxidation levels, activation marker expressions, GPIb/ɑ desialylation with exposure of glycan residues and other quality parameters of platelets were measured and compared between the groups. Phagocytosis of platelets was detected by phorbol 12-myristate 13-acetate-activated THP-1 or Hep G2 cells. Moreover, the recovery of infused platelets was measured in severe combined immunodeficient mice at different timepoints.</p><p><strong>Results: </strong>After 5 days of storage, cytoplasmic reactive oxygen species significantly increased in chilled compared to non-chilled platelets; they were notably reduced with the addition of N-acetylcysteine, particularly at a concentration of 5 mM. Compared with chilled platelets, the P-selectin and phosphatidylserine expressions, as well as exposure of GPIb/ɑ glycan residues, were significantly reduced with 5 mM of N-acetylcysteine. Phagocytosis of platelets by THP-1 or Hep G2 cells was significantly lower in 5 mM of N-acetylcysteine compared to cold-stored platelets without N-acetylcysteine.</p><p><strong>Conclusions: </strong>This study demonstrated correlations between reactive oxygen species production and their pro-oxidant effects on platelet clearance after cold storage. The addition of N-acetylcysteine at an appropriate concentration do not only protects chilled platelets from storage lesions caused by reactive oxygen species overproduction but also prevents platelet phagocytosis in vitro and clearance in vivo, thereby extending circulating time.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142585411","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 : 2024-10-18DOI: 10.1016/j.htct.2024.09.2478
Laila M Sherief, Mohamed Beshir, Sahar N Saleem, Wesam Elmozy, Mona Elkalioubie, Basma K Soliman, Amr M Fawzy, Mona Alsharkawy, Diana Hanna
Introduction: Childhood acute lymphoblastic leukemia survivors receiving multiple packed red blood transfusions may be at risk of vital organ iron deposition causing long-term complications. This study was undertaken to assess the prevalence and severity of iron overload in the liver and heart by magnetic resonance imaging.
Methods: A case-control study was conducted on 60 acute lymphoblastic leukemia survivors aged from 6 to 18 years and 60 healthy, age- and sex-matched children as a control group. The hematological profile, and serum ferritin was assessed and the iron content of the liver and heart was measured by T2* magnetic resonance imaging.
Results: Twenty-six (43.3 %) and two (3.3 %) patients had elevated liver and myocardial iron concentrations, respectively. The statistics show a significantly positive correlation between liver T2* magnetic resonance and serum ferritin. The total volume of blood transfused and duration of follow up were associated with elevated liver iron concentrations (p-values = 0.036 and 0.028 respectively). Myocardial T2* magnetic resonance lacked correlation with serum ferritin and transfusion therapy CONCLUSION: Liver iron overload was detected in children and adolescents after acute lymphoblastic leukemia therapy. The risk of iron overload was related mainly to the transfusion burden during therapy. These patients need monitoring after therapy to assess their need for future chelation therapy.
{"title":"Assessment of transfusion-induced iron overload with T2*MRI in survivors of childhood acute lymphoblastic leukemia: A case control study.","authors":"Laila M Sherief, Mohamed Beshir, Sahar N Saleem, Wesam Elmozy, Mona Elkalioubie, Basma K Soliman, Amr M Fawzy, Mona Alsharkawy, Diana Hanna","doi":"10.1016/j.htct.2024.09.2478","DOIUrl":"https://doi.org/10.1016/j.htct.2024.09.2478","url":null,"abstract":"<p><strong>Introduction: </strong>Childhood acute lymphoblastic leukemia survivors receiving multiple packed red blood transfusions may be at risk of vital organ iron deposition causing long-term complications. This study was undertaken to assess the prevalence and severity of iron overload in the liver and heart by magnetic resonance imaging.</p><p><strong>Methods: </strong>A case-control study was conducted on 60 acute lymphoblastic leukemia survivors aged from 6 to 18 years and 60 healthy, age- and sex-matched children as a control group. The hematological profile, and serum ferritin was assessed and the iron content of the liver and heart was measured by T2* magnetic resonance imaging.</p><p><strong>Results: </strong>Twenty-six (43.3 %) and two (3.3 %) patients had elevated liver and myocardial iron concentrations, respectively. The statistics show a significantly positive correlation between liver T2* magnetic resonance and serum ferritin. The total volume of blood transfused and duration of follow up were associated with elevated liver iron concentrations (p-values = 0.036 and 0.028 respectively). Myocardial T2* magnetic resonance lacked correlation with serum ferritin and transfusion therapy CONCLUSION: Liver iron overload was detected in children and adolescents after acute lymphoblastic leukemia therapy. The risk of iron overload was related mainly to the transfusion burden during therapy. These patients need monitoring after therapy to assess their need for future chelation therapy.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142570803","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}
Background: Acute lymphoblastic leukemia is the predominant neoplastic ailment in childhood. Prior research has already established noteworthy connections between CDKN2A polymorphisms and susceptibility to this childhood leukemia, however, substantial associations are still awaiting validation. This investigation was undertaken to examine the correlation between CDKN2A polymorphisms and the risk of acute lymphoblastic leukemia in children.
Methods: Acquisition of information encompassed the exploration of diverse databases including PubMed, Scopus, EMBASE, and China National Knowledge Infrastructure (CNKI) until January 10, 2024. An estimation of associations was achieved utilizing odds ratios with 95% confidence intervals.
Results: A total of 22 case-control studies encompassing 10,203 cases of acute lymphoblastic leukemia and 36,424 healthy controls were included. Within this pool of studies, 14 focused on rs3731217, comprising 5396 cases and 15,787 controls, whereas eight studies investigated rs3731249, comprising 4807 cases and 20,637 controls. The aggregated data showed that the rs3731217 variant offers protection against acute lymphoblastic leukemia. Nevertheless, when subgroups are analyzed according to ethnicity, it becomes clear that the rs3731217 polymorphism significantly influences susceptibility, particularly among individuals of Caucasian and African descent with no such association being observed in children of Asian origin. Nevertheless, the rs3731249 polymorphism displayed a noteworthy correlation with vulnerability to pediatric acute lymphoblastic leukemia.
Conclusion: The aggregated data revealed that the rs3731217 variation offers protection against the development of pediatric acute lymphoblastic leukemia and the rs3731249 polymorphism is significantly correlated with susceptibility.
{"title":"A comprehensive consolidation of data on the connection between CDKN2A polymorphisms and the susceptibility to childhood acute lymphoblastic leukemia.","authors":"Maryam Aghasipour, Fatemeh Asadian, Seyed Alireza Dastgheib, Abolhasan Alijanpour, Ali Masoudi, Maedeh Barahman, Mohammad Golshan-Tafti, Reza Bahrami, Amirmasoud Shiri, Hossein Aarafi, Kazem Aghili, Hossein Neamatzadeh","doi":"10.1016/j.htct.2024.05.017","DOIUrl":"https://doi.org/10.1016/j.htct.2024.05.017","url":null,"abstract":"<p><strong>Background: </strong>Acute lymphoblastic leukemia is the predominant neoplastic ailment in childhood. Prior research has already established noteworthy connections between CDKN2A polymorphisms and susceptibility to this childhood leukemia, however, substantial associations are still awaiting validation. This investigation was undertaken to examine the correlation between CDKN2A polymorphisms and the risk of acute lymphoblastic leukemia in children.</p><p><strong>Methods: </strong>Acquisition of information encompassed the exploration of diverse databases including PubMed, Scopus, EMBASE, and China National Knowledge Infrastructure (CNKI) until January 10, 2024. An estimation of associations was achieved utilizing odds ratios with 95% confidence intervals.</p><p><strong>Results: </strong>A total of 22 case-control studies encompassing 10,203 cases of acute lymphoblastic leukemia and 36,424 healthy controls were included. Within this pool of studies, 14 focused on rs3731217, comprising 5396 cases and 15,787 controls, whereas eight studies investigated rs3731249, comprising 4807 cases and 20,637 controls. The aggregated data showed that the rs3731217 variant offers protection against acute lymphoblastic leukemia. Nevertheless, when subgroups are analyzed according to ethnicity, it becomes clear that the rs3731217 polymorphism significantly influences susceptibility, particularly among individuals of Caucasian and African descent with no such association being observed in children of Asian origin. Nevertheless, the rs3731249 polymorphism displayed a noteworthy correlation with vulnerability to pediatric acute lymphoblastic leukemia.</p><p><strong>Conclusion: </strong>The aggregated data revealed that the rs3731217 variation offers protection against the development of pediatric acute lymphoblastic leukemia and the rs3731249 polymorphism is significantly correlated with susceptibility.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142515298","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}
Background: Sickle cell disease is the most common inherited blood disorder in the world with the birth of approximately 300,000 newborns screened each year. In 2009, the World Health Organization ranked the fight against sickle cell disease among the priorities for the Africa regions. The best way to prevent this incurable disease remains, on one hand systematic screening at birth, and on the other the proscription of risky union between heterozygous subjects.
Aim: The aim of this study was to analyze the epidemiological profile of sickle cell disease and other hemoglobinopathies in Benin and determine more up-to-date prevalence rates of the disease within the population.
Methods: The hemoglobin profiles of 2910 study participants were determined by quantitative electrophoresis. Samples with abnormal hemoglobin results were subjected to a complete blood count.
Results: Our study population was balanced between males (1528) and females (1382) with a sex ratio of 1.1. The mean age ranged from eight years in the pediatric group to 26 years in adults. The hemoglobin electrophoresis profiles found were as follows: 59.7 % Hb AA (normal), 21.7 % Hb AS, 10.2 % Hb AC, 3.1 % Hb SS, 3.7 % Hb SC, and 1.6 % of the rare phenotypes (Hb AD, Hb AE, Hb AF, Hb A/β-thal, Hb SD, Hb SF, Hb CC and Hb C/β-thal). Participants with abnormal hemoglobin presented a normochromic normocytic anemia. A total of 356 (12 %) people knew their profile compared to 2554 (88 %) who did not.
Conclusion: The high prevalence of hemoglobinopathies found in this study highlights in importance of screening in the Benin population.
{"title":"Epidemiologic profile of hemoglobinopathies in Benin.","authors":"Selma Gomez, Adjile Edjide Roukiyath Amoussa, Edwige Dedjinou, Manasse Kakpo, Pélagie Gbédji, Nouhoum Amossou Soulé, Bernice Quenum","doi":"10.1016/j.htct.2024.07.008","DOIUrl":"https://doi.org/10.1016/j.htct.2024.07.008","url":null,"abstract":"<p><strong>Background: </strong>Sickle cell disease is the most common inherited blood disorder in the world with the birth of approximately 300,000 newborns screened each year. In 2009, the World Health Organization ranked the fight against sickle cell disease among the priorities for the Africa regions. The best way to prevent this incurable disease remains, on one hand systematic screening at birth, and on the other the proscription of risky union between heterozygous subjects.</p><p><strong>Aim: </strong>The aim of this study was to analyze the epidemiological profile of sickle cell disease and other hemoglobinopathies in Benin and determine more up-to-date prevalence rates of the disease within the population.</p><p><strong>Methods: </strong>The hemoglobin profiles of 2910 study participants were determined by quantitative electrophoresis. Samples with abnormal hemoglobin results were subjected to a complete blood count.</p><p><strong>Results: </strong>Our study population was balanced between males (1528) and females (1382) with a sex ratio of 1.1. The mean age ranged from eight years in the pediatric group to 26 years in adults. The hemoglobin electrophoresis profiles found were as follows: 59.7 % Hb AA (normal), 21.7 % Hb AS, 10.2 % Hb AC, 3.1 % Hb SS, 3.7 % Hb SC, and 1.6 % of the rare phenotypes (Hb AD, Hb AE, Hb AF, Hb A/β-thal, Hb SD, Hb SF, Hb CC and Hb C/β-thal). Participants with abnormal hemoglobin presented a normochromic normocytic anemia. A total of 356 (12 %) people knew their profile compared to 2554 (88 %) who did not.</p><p><strong>Conclusion: </strong>The high prevalence of hemoglobinopathies found in this study highlights in importance of screening in the Benin population.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484128","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 : 2024-09-25DOI: 10.1016/j.htct.2024.07.007
Astrid Pavlovsky, Juan Ignacio Garcia Altuve, Amalia Cerutti, Lorena Fiad, Nicolás Kurgansky, Fernando Warley, Florencia Negri Aranguren
Introduction: The BV-AVD (Brentuximab vedotin, doxorubicin, vinblastine, and dacarbazine) combination for first-line treatment of advanced stage Hodgkin's lymphoma has been approved by regulatory authorities and included in international guidelines. However, several factors influence its incorporation as standard of care.
Materials and methods: A group of experts from different institutions was identified and, using the Delphi method, an analysis of the results of the ECHELON 1 trial for the indication of BV-AVD over ABVD (doxorubicin hydrochloride, bleomycin sulfate, vinblastine sulfate, dacarbazine) in patients with Hodgkin's lymphoma Stages III and IV in Argentina was done. The clinical and academic experience of the authors and the context of the Argentine healthcare system were considered.
Results and discussion: Seven statements on general aspects of the management of Hodgkin's lymphoma and nine on specific aspects related to the use of BV-AVD over ABVD reached a consensus of agreement. There was a strong expert consensus in favor of indicating BV-AVD in the presence of extranodal disease or pulmonary disease. Moderate to severe neuropathy, pregnancy and drug allergy were considered absolute contraindications to prescribe BV.
Conclusions: The authors agreed that BV-AVD could be considered a new treatment option in high-risk patients. However health system-dependent factors (such as high cost, lack of availability, reimbursement difficulties, irregular delivery, and issues with granulocyte-colony stimulating factor availability) could pose limitations for this prescription. While awaiting new data from clinical trials and real-world studies, these recommendations can represent a useful tool for hematologists in different parts of the world.
{"title":"Benefits of BV-AVD (Brentuximab vedotin, doxorubicin, vinblastine, and dacarbazine) versus ABVD (doxorubicin hydrochloride, bleomycin sulfate, vinblastine sulfate, dacarbazine) in patients with advanced-stage Hodgkin's lymphoma: an analysis of the ECHELON 1 trial by the GATLA group using the Delphi Method.","authors":"Astrid Pavlovsky, Juan Ignacio Garcia Altuve, Amalia Cerutti, Lorena Fiad, Nicolás Kurgansky, Fernando Warley, Florencia Negri Aranguren","doi":"10.1016/j.htct.2024.07.007","DOIUrl":"10.1016/j.htct.2024.07.007","url":null,"abstract":"<p><strong>Introduction: </strong>The BV-AVD (Brentuximab vedotin, doxorubicin, vinblastine, and dacarbazine) combination for first-line treatment of advanced stage Hodgkin's lymphoma has been approved by regulatory authorities and included in international guidelines. However, several factors influence its incorporation as standard of care.</p><p><strong>Materials and methods: </strong>A group of experts from different institutions was identified and, using the Delphi method, an analysis of the results of the ECHELON 1 trial for the indication of BV-AVD over ABVD (doxorubicin hydrochloride, bleomycin sulfate, vinblastine sulfate, dacarbazine) in patients with Hodgkin's lymphoma Stages III and IV in Argentina was done. The clinical and academic experience of the authors and the context of the Argentine healthcare system were considered.</p><p><strong>Results and discussion: </strong>Seven statements on general aspects of the management of Hodgkin's lymphoma and nine on specific aspects related to the use of BV-AVD over ABVD reached a consensus of agreement. There was a strong expert consensus in favor of indicating BV-AVD in the presence of extranodal disease or pulmonary disease. Moderate to severe neuropathy, pregnancy and drug allergy were considered absolute contraindications to prescribe BV.</p><p><strong>Conclusions: </strong>The authors agreed that BV-AVD could be considered a new treatment option in high-risk patients. However health system-dependent factors (such as high cost, lack of availability, reimbursement difficulties, irregular delivery, and issues with granulocyte-colony stimulating factor availability) could pose limitations for this prescription. While awaiting new data from clinical trials and real-world studies, these recommendations can represent a useful tool for hematologists in different parts of the world.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142376479","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 : 2024-09-23DOI: 10.1016/j.htct.2024.07.006
Lauro Fabián Amador-Medina, Erick Crespo-Solís, Francisco Javier Turrubiates-Hernández, Karla Edith Santibañez-Bedolla
Background: Based on the VIALE-A and VIALE-C studies, the Food and Drug Administration approved venetoclax in 2020 in combination with azacitidine or low-dose cytarabine for the treatment of patients with acute myeloid leukemia ineligible for intensive chemotherapy. After the publication of these studies, venetoclax/azacitidine was assumed to be superior to venetoclax/low-dose cytarabine; however, these studies were not designed to demonstrate superiority between these combinations. Therefore, we conducted a systematic review to describe overall survival, complete remission rate, and composite complete remission rate to assess response of these two regimens in patients with newly diagnosed acute myeloid leukemia who are ineligible for intensive chemotherapy.
Materials and methods: The PubMed and Web of Science databases were searched for retrospective studies and complete remission, composite complete remission, and overall survival rates were recorded.
Results: Only 11 of the 815 publications identified were eligible to be included n this review, ten studies evaluated the venetoclax/azacitidine combination and one study evaluated the venetoclax/low-dose cytarabine combination. The median overall survival for venetoclax/azacitidine was 10.75 months, whereas for venetoclax/low-dose cytarabine the median overall survival had not been reached at the time of publication. Composite complete remission was 63.3 % for venetoclax/azacitidine and 90 % for venetoclax/low-dose cytarabine. Adverse events were similar for both combinations.
Conclusions: A limited number of studies investigating the venetoclax/low-dose cytarabine combination exist. Based on the available data, the superiority of venetoclax/azacitidine over venetoclax/low-dose cytarabine cannot be assumed for all acute myeloid leukemia patients who are ineligible for intensive chemotherapy. Venetoclax/low-dose cytarabine can still be considered as an option for the drug combinations currently under investigation.
{"title":"Venetoclax with low-dose cytarabine, a forgotten combination in patients with acute myeloid leukemia ineligible for intensive chemotherapy: a systematic review.","authors":"Lauro Fabián Amador-Medina, Erick Crespo-Solís, Francisco Javier Turrubiates-Hernández, Karla Edith Santibañez-Bedolla","doi":"10.1016/j.htct.2024.07.006","DOIUrl":"10.1016/j.htct.2024.07.006","url":null,"abstract":"<p><strong>Background: </strong>Based on the VIALE-A and VIALE-C studies, the Food and Drug Administration approved venetoclax in 2020 in combination with azacitidine or low-dose cytarabine for the treatment of patients with acute myeloid leukemia ineligible for intensive chemotherapy. After the publication of these studies, venetoclax/azacitidine was assumed to be superior to venetoclax/low-dose cytarabine; however, these studies were not designed to demonstrate superiority between these combinations. Therefore, we conducted a systematic review to describe overall survival, complete remission rate, and composite complete remission rate to assess response of these two regimens in patients with newly diagnosed acute myeloid leukemia who are ineligible for intensive chemotherapy.</p><p><strong>Materials and methods: </strong>The PubMed and Web of Science databases were searched for retrospective studies and complete remission, composite complete remission, and overall survival rates were recorded.</p><p><strong>Results: </strong>Only 11 of the 815 publications identified were eligible to be included n this review, ten studies evaluated the venetoclax/azacitidine combination and one study evaluated the venetoclax/low-dose cytarabine combination. The median overall survival for venetoclax/azacitidine was 10.75 months, whereas for venetoclax/low-dose cytarabine the median overall survival had not been reached at the time of publication. Composite complete remission was 63.3 % for venetoclax/azacitidine and 90 % for venetoclax/low-dose cytarabine. Adverse events were similar for both combinations.</p><p><strong>Conclusions: </strong>A limited number of studies investigating the venetoclax/low-dose cytarabine combination exist. Based on the available data, the superiority of venetoclax/azacitidine over venetoclax/low-dose cytarabine cannot be assumed for all acute myeloid leukemia patients who are ineligible for intensive chemotherapy. Venetoclax/low-dose cytarabine can still be considered as an option for the drug combinations currently under investigation.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142376481","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}