The advantages of albumin over less costly alternative fluids continue to be debated. Many scientific articles were devoted to the clinical analysis of the use of albumin in acute illness as well as its comparison with other fluid regimens. However, the lack of fundamental knowledge about the physical and chemical properties of commercial albumin generates many unpromising discussions about the effectiveness of the use of albumin among practitioners and medical scientists. The manuscript provides information about the different variants of commercial albumin, the mechanisms of their action, indications and contraindications to use. The presented information is based on fundamental knowledge of physical and chemical properties of commercial albumin. This manuscript is not only educational information, but also is guide to action for clinicians.
{"title":"What Clinicians Should Know About the Use of Albumin","authors":"A. Belousov","doi":"10.23880/hij-16000139","DOIUrl":"https://doi.org/10.23880/hij-16000139","url":null,"abstract":"The advantages of albumin over less costly alternative fluids continue to be debated. Many scientific articles were devoted to the clinical analysis of the use of albumin in acute illness as well as its comparison with other fluid regimens. However, the lack of fundamental knowledge about the physical and chemical properties of commercial albumin generates many unpromising discussions about the effectiveness of the use of albumin among practitioners and medical scientists. The manuscript provides information about the different variants of commercial albumin, the mechanisms of their action, indications and contraindications to use. The presented information is based on fundamental knowledge of physical and chemical properties of commercial albumin. This manuscript is not only educational information, but also is guide to action for clinicians.","PeriodicalId":245976,"journal":{"name":"Haematology International Journal","volume":"6 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125370074","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: The −158 (C→T) nucleotide change, known as Xmn I polymorphism, occurs in G γ-globin gene promoter and may result in elevated fetal hemoglobin (HbF). However some studies did not find any association of HbF with the mutation. This study was taken to confirm the influence of Xmn-I polymorphism on HbF production in thalassemia intermedia patients in Pakistani population. Methods: Blood samples from 100 known thalassemia intermedia patients were collected and analyzed for Xmn -I polymorphism and levels of hemoglobin F. Results: High levels of HbF were found in Thalassemia intermedia patients being heterozygous and homozygous for Xmn -I polymorphism. Conclusion: Xmn I polymorphism (C-T) of the G γ-globin gene promoter is associated with increased expression of the G γ-globin gene, higher production of HbF and lesser clinical severity.
{"title":"Contribution of Xmn I Polymorphism in the Variation of Hemoglobin F in Thalassemia Intermedia Patients in Pakistan","authors":"A. S","doi":"10.23880/hij-16000179","DOIUrl":"https://doi.org/10.23880/hij-16000179","url":null,"abstract":"Background: The −158 (C→T) nucleotide change, known as Xmn I polymorphism, occurs in G γ-globin gene promoter and may result in elevated fetal hemoglobin (HbF). However some studies did not find any association of HbF with the mutation. This study was taken to confirm the influence of Xmn-I polymorphism on HbF production in thalassemia intermedia patients in Pakistani population. Methods: Blood samples from 100 known thalassemia intermedia patients were collected and analyzed for Xmn -I polymorphism and levels of hemoglobin F. Results: High levels of HbF were found in Thalassemia intermedia patients being heterozygous and homozygous for Xmn -I polymorphism. Conclusion: Xmn I polymorphism (C-T) of the G γ-globin gene promoter is associated with increased expression of the G γ-globin gene, higher production of HbF and lesser clinical severity.","PeriodicalId":245976,"journal":{"name":"Haematology International Journal","volume":"56 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125506638","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}
{"title":"Peripheral T Cell Lymphoma with Follicular Helper Phenotype, Case Report and Literature Review","authors":"Katty Ar","doi":"10.23880/HIJ-16000135","DOIUrl":"https://doi.org/10.23880/HIJ-16000135","url":null,"abstract":"","PeriodicalId":245976,"journal":{"name":"Haematology International Journal","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129854458","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}
American Society of Hematology (ASH) recommends antileukemic therapy over best supportive care for older adults with acute myeloid leukemia (AML) [1]. According to the SEER database from 2010 to 2017, the 5-year overall survival (OS) in individuals with AML diagnosis over 70 years of age was around 5% [2]. We describe a case of nearly one-year survival in an elderly patient with a very high-risk myelodysplastic syndrome (MDS) that later transformed into AML with supportive therapy only.
{"title":"Case Report of Successful Symptomatic Management of Elderly Patient with Very High-Risk MDS-Transformed AML","authors":"Hakobyan Y","doi":"10.23880/hij-16000191","DOIUrl":"https://doi.org/10.23880/hij-16000191","url":null,"abstract":"American Society of Hematology (ASH) recommends antileukemic therapy over best supportive care for older adults with acute myeloid leukemia (AML) [1]. According to the SEER database from 2010 to 2017, the 5-year overall survival (OS) in individuals with AML diagnosis over 70 years of age was around 5% [2]. We describe a case of nearly one-year survival in an elderly patient with a very high-risk myelodysplastic syndrome (MDS) that later transformed into AML with supportive therapy only.","PeriodicalId":245976,"journal":{"name":"Haematology International Journal","volume":"76 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126905068","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}
{"title":"Ambient or Static-T lymphoblastic Leukaemia/Lymphoma","authors":"Anu Bajaj","doi":"10.23880/hij-16000211","DOIUrl":"https://doi.org/10.23880/hij-16000211","url":null,"abstract":"","PeriodicalId":245976,"journal":{"name":"Haematology International Journal","volume":"629 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116472135","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}
Thalassemia is common inherited disorder among humans, and they represent a major public health problem in many areas of the world. The study aimed to the measurement of hematological characterization of beta-thalassemia in Sudanese patients. Blood samples from 61 beta-thalassemic patients were collected after written consent form obtained from all participants. The frequency of adults (>18 years) was 45 (73.8%) and children’s (<18 years) was 16 (26.2%); the frequency of male was 27 (44.3%) and 34 were female (55.7%). Hemoglobin estimation and red cell indices were carried out using the automatic blood cell counter Sysmex K × 21N. The results showed that Hb and RBCs indices were varied between mild to moderate and severe decreasing, hemoglobin concentration (Hb) with the mean value of 9.6 g/dL, with minimum value of 6.1 g/dl and maximum of 11.9 g/dl, while RBCs were increased in all patients, mean value 5.2 c/l, mean corpuscular volume mean was 58.9 fl, hematocrit was 30.4, mean corpuscular hemoglobin (MCH) 18.8 pg, mean corpuscular hemoglobin concentration (MCHC) was 31.7pg, and RDW was 18.8%. The method used for hemoglobin electrophoresis was capillary electrophoresis, Hb pattern shows increased HbA2 and HbF, the mean of HbA is 78.3%, HbF is 2.3%, and HbA 2 is 6.5% with the min. value of 3.6% and max. of 12.2%. While the mean of serum iron was 82.75 showed low level, 19 high level, and 35 were normal level. Comparison of hematological analysis (HbA2) in thalassemic patients coexisted with iron deficiency and without result was insignificant difference (p=0.645), this result disagrees with references that say iron deficiency masking HbA2. Nevertheless, the association between HbA2 and HbF revealed a statistically significant difference (p<0.013) and HbA2 with Hb was insignificant (p=0.260). ABSTRACT Thalassemia is a Mendelian autosomal recessive heritable blood disorder, it is a group of genetically determined microcytic, hypochromic anemia’s resulting from a decrease in synthesis of one or more globin chains in the hemoglobin molecule [4]. The most common types are alpha- and beta-thalassemia according to which globin chain is reduced [15]. Beta-thalassemia is classified into three types depending on the severity of symptoms: Thalassemia major also known as Cooley’s anemia [8,16]. Thalassemia intermediate and thalassemia minor, thalassemia major is more severe. The signs and symptoms of thalassemia major appear within the first 2 years of life, children develop life-threatening anemia, and they do not gain weight and grow at the expected rate (failure to thrive) and may develop yellowing of the skin and whites of the eyes (jaundice) [18]. Affected individuals may have an enlarged spleen, liver, heart, and their bones which may be misshapen. adolescents thalassemia major delayed puberty.
{"title":"Hematological Characterization of Beta Thalassemia in Sudanese Patients","authors":"Rabab Hassan Elshaikh","doi":"10.23880/hij-16000150","DOIUrl":"https://doi.org/10.23880/hij-16000150","url":null,"abstract":"Thalassemia is common inherited disorder among humans, and they represent a major public health problem in many areas of the world. The study aimed to the measurement of hematological characterization of beta-thalassemia in Sudanese patients. Blood samples from 61 beta-thalassemic patients were collected after written consent form obtained from all participants. The frequency of adults (>18 years) was 45 (73.8%) and children’s (<18 years) was 16 (26.2%); the frequency of male was 27 (44.3%) and 34 were female (55.7%). Hemoglobin estimation and red cell indices were carried out using the automatic blood cell counter Sysmex K × 21N. The results showed that Hb and RBCs indices were varied between mild to moderate and severe decreasing, hemoglobin concentration (Hb) with the mean value of 9.6 g/dL, with minimum value of 6.1 g/dl and maximum of 11.9 g/dl, while RBCs were increased in all patients, mean value 5.2 c/l, mean corpuscular volume mean was 58.9 fl, hematocrit was 30.4, mean corpuscular hemoglobin (MCH) 18.8 pg, mean corpuscular hemoglobin concentration (MCHC) was 31.7pg, and RDW was 18.8%. The method used for hemoglobin electrophoresis was capillary electrophoresis, Hb pattern shows increased HbA2 and HbF, the mean of HbA is 78.3%, HbF is 2.3%, and HbA 2 is 6.5% with the min. value of 3.6% and max. of 12.2%. While the mean of serum iron was 82.75 showed low level, 19 high level, and 35 were normal level. Comparison of hematological analysis (HbA2) in thalassemic patients coexisted with iron deficiency and without result was insignificant difference (p=0.645), this result disagrees with references that say iron deficiency masking HbA2. Nevertheless, the association between HbA2 and HbF revealed a statistically significant difference (p<0.013) and HbA2 with Hb was insignificant (p=0.260). ABSTRACT Thalassemia is a Mendelian autosomal recessive heritable blood disorder, it is a group of genetically determined microcytic, hypochromic anemia’s resulting from a decrease in synthesis of one or more globin chains in the hemoglobin molecule [4]. The most common types are alpha- and beta-thalassemia according to which globin chain is reduced [15]. Beta-thalassemia is classified into three types depending on the severity of symptoms: Thalassemia major also known as Cooley’s anemia [8,16]. Thalassemia intermediate and thalassemia minor, thalassemia major is more severe. The signs and symptoms of thalassemia major appear within the first 2 years of life, children develop life-threatening anemia, and they do not gain weight and grow at the expected rate (failure to thrive) and may develop yellowing of the skin and whites of the eyes (jaundice) [18]. Affected individuals may have an enlarged spleen, liver, heart, and their bones which may be misshapen. adolescents thalassemia major delayed puberty.","PeriodicalId":245976,"journal":{"name":"Haematology International Journal","volume":"47 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126168628","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}
Thrombocytopenia is a common hematological abnormality in newly diagnosed acute myeloid leukemia (AML) which may be linked to the degree of marrow infiltration by leukemic blasts as well as altered cytokine milieu in a subset of AMLs. We report the presence of circulating megakaryocytes in peripheral blood as a sign of satisfactory platelet recovery post induction chemotherapy in a pediatric subject with a favourable risk WHO category I AML with synchronous extramedullary myeloid cell tumor; and present a brief review of literature on the role of platelet recovery in AML prognosis.
{"title":"Circulating Megakaryocytes in Peripheral Blood: A Clue to Satisfactory Platelet Recovery Post Induction Chemotherapy in Acute Myeloid Leukemia","authors":"S. Padhi","doi":"10.23880/hij-16000198","DOIUrl":"https://doi.org/10.23880/hij-16000198","url":null,"abstract":"Thrombocytopenia is a common hematological abnormality in newly diagnosed acute myeloid leukemia (AML) which may be linked to the degree of marrow infiltration by leukemic blasts as well as altered cytokine milieu in a subset of AMLs. We report the presence of circulating megakaryocytes in peripheral blood as a sign of satisfactory platelet recovery post induction chemotherapy in a pediatric subject with a favourable risk WHO category I AML with synchronous extramedullary myeloid cell tumor; and present a brief review of literature on the role of platelet recovery in AML prognosis.","PeriodicalId":245976,"journal":{"name":"Haematology International Journal","volume":"155 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129586913","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}
{"title":"Agranulocytosis – Causes, Clinical Significance, Management -A Mini Review","authors":"Dudani S","doi":"10.23880/hij-16000195","DOIUrl":"https://doi.org/10.23880/hij-16000195","url":null,"abstract":"","PeriodicalId":245976,"journal":{"name":"Haematology International Journal","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130775134","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}
COVID-19 pandemia is a major health emergency causing hundreds of deaths worldwide. The high reported morbidity has been related to derangement of the immune system, cytokine overproduction and endothelial dysfunction. Several drugs currently marketed for blood disorders are being tested for potential clinical benefit in patients with COVID-19 and hematologists are valuable partners for multidisciplinary research projects assessing host-targeted therapies. This review attempts to highlight the pathogenesis of lymphopenia in COVID-19 disease and the possible treatment pathways. While discussing the pattern of cytokine hyperproduction, we also focused on anti-cytokine drugs being tested for COVID-19 disease but currently prescribed for blood disorders.
{"title":"COVID-19-Driven Immunoparalysis and Cytokine Storm: Can Hematologists Contribute to the Battle?","authors":"Marchetti M","doi":"10.23880/hij-16000170","DOIUrl":"https://doi.org/10.23880/hij-16000170","url":null,"abstract":"COVID-19 pandemia is a major health emergency causing hundreds of deaths worldwide. The high reported morbidity has been related to derangement of the immune system, cytokine overproduction and endothelial dysfunction. Several drugs currently marketed for blood disorders are being tested for potential clinical benefit in patients with COVID-19 and hematologists are valuable partners for multidisciplinary research projects assessing host-targeted therapies. This review attempts to highlight the pathogenesis of lymphopenia in COVID-19 disease and the possible treatment pathways. While discussing the pattern of cytokine hyperproduction, we also focused on anti-cytokine drugs being tested for COVID-19 disease but currently prescribed for blood disorders.","PeriodicalId":245976,"journal":{"name":"Haematology International Journal","volume":"21 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126259953","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}
{"title":"Red Cell Autoantibodies in a Thalassemia Major Patient","authors":"B. Nidhi","doi":"10.23880/hij-16000145","DOIUrl":"https://doi.org/10.23880/hij-16000145","url":null,"abstract":"","PeriodicalId":245976,"journal":{"name":"Haematology International Journal","volume":"21 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122323136","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}
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