M. B. Deepak, S. Bharath Ram, S. Chougule, Hema Subramanian, K. S. Shalini, S. Damodar
{"title":"Acute Myeloid Leukemia t (8;21) with Masked Systemic Mastocytosis","authors":"M. B. Deepak, S. Bharath Ram, S. Chougule, Hema Subramanian, K. S. Shalini, S. Damodar","doi":"10.1007/s12288-024-01733-x","DOIUrl":"https://doi.org/10.1007/s12288-024-01733-x","url":null,"abstract":"","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139867015","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Potential Role of Positivity Percentage of CD200 in the Mature B Cell Neoplasms","authors":"Can Yan, Weichu Dai, Na Shi, Wei Pan","doi":"10.1007/s12288-023-01727-1","DOIUrl":"https://doi.org/10.1007/s12288-023-01727-1","url":null,"abstract":"","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-01-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139383797","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Potential Clinical Role of LncRNA miR503HG in Multiple Myeloma and its Effect on the Proliferation and Adhesion of Myeloma Cells.","authors":"Pingling Yin, Xiang Zhou","doi":"10.1007/s12288-023-01658-x","DOIUrl":"10.1007/s12288-023-01658-x","url":null,"abstract":"<p><p>This study mainly explored the role of lncRNA miR503HG in multiple myeloma and the potential downstream regulatory mechanism affecting disease. Real-time quantitative polymerase chain reaction was used to measure the expression levels of miR503HG and miR-103. A cell counting kit-8 assay was performed to detect cell viability. The concentrations of adhesion-related factors (MUC-1, VCAM-1, ICAM-1) were determined using enzyme-linked immunosorbent assay. The targeting relationship between miR503HG and miR-103 was detected by dual-luciferase reporter assay. The miR503HG expression in peripheral blood of multiple myeloma patients was lower than that of normal healthy individuals and associated with ISS stage and worse overall survival. miR-103 was identified as the downstream target of miR503HG. Upregulation of miR503HG could inhibit cell proliferation and adhesion of multiple myeloma cell lines, which could partially reverse the inhibition of adhesion and proliferation by high expression of miR-103. lncRNA miR503HG expression was downregulated in multiple myeloma and had potential diagnostic/prognostic value. MiR503HG exerts a molecular sponge effect on miR-103 and affects its expression, thus achieving the inhibitory effect on multiple myeloma.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10830954/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45509259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hari Menon, Pawan Kumar Singh, Bhausaheb Bagal, Tuphan Dolai, Ankita Jain, Antara Chaudhri
{"title":"Minimal Residual Disease in the Management of B-Cell Acute Lymphoblastic Leukemia: A Systematic Review of Studies from Indian Settings.","authors":"Hari Menon, Pawan Kumar Singh, Bhausaheb Bagal, Tuphan Dolai, Ankita Jain, Antara Chaudhri","doi":"10.1007/s12288-023-01641-6","DOIUrl":"10.1007/s12288-023-01641-6","url":null,"abstract":"<p><p>Minimal residual disease (MRD) has become an essential tool in the management of B-cell acute lymphoblastic leukemia (B-ALL) and aids in tailoring treatment strategies to suit specific patient needs. Although much progress has been made in this area, there is limited data on the use of MRD in the Indian context. Our objective was to identify relevant literature that discusses the utility of MRD in the management of B-cell ALL in adolescents and young adults (AYA) and adults in Indian settings. A systematic search and screening of articles were performed using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. The primary data source was PubMed followed by Google Scholar for articles and conference proceedings. Of the 254 records screened, 24 records were retained for analysis. MRD monitoring had a significant role in the management of AYA/adult B-cell ALL patients. Variability of results was observed across these studies with respect to methods, techniques, and use. However, these studies evidenced and validated the importance of MRD assessment in risk-adapted management of B-cell ALL and highlighted the need for optimization. The advances in MRD diagnostics and applications are yet to be tested and adopted in Indian settings. Hence, there is a need for in-depth research to develop and optimize approaches for calibrating country-specific management strategies. The potential role of MRD assessments in anticipating relapse or treatment failures warrants more attention for the preemptive positioning of novel strategies involving immunotherapies.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10831037/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49207068","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rhythm Joshi, Aakriti Garg, Zehva Khan, Dinesh Bhurani, Nidhi, Mohd Ashif Khan
{"title":"Short-Term Impact of Hematopoietic Stem Cell Transplantation on Depressive Behavior, Cognition and Quality of Life in Leukemia Patients.","authors":"Rhythm Joshi, Aakriti Garg, Zehva Khan, Dinesh Bhurani, Nidhi, Mohd Ashif Khan","doi":"10.1007/s12288-023-01653-2","DOIUrl":"10.1007/s12288-023-01653-2","url":null,"abstract":"<p><p>Hematopoietic stem cell transplantation (HSCT) or Bone Marrow Transplantation (BMT) has significantly improved the survival rates of patients suffering from hematological malignancies. However, the cure can only be achieved at the price of morbidity and long-term complications. Thus, this study aimed to evaluate the short-term effect of HSCT on depressive behavior, cognition, and quality of life (QoL) in leukemia patients. Sixty patients were included in this prospective observational study. The current study assessed depression using Patient Health Questionnaire (PHQ-9) scale, cognition using Montreal Cognitive Assessment (MOCA) scale and QoL using European Organization for the Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ C30) before 7 days of the therapy i.e., preconditioning/baseline (TP1) and after 30 days of the treatment (TP2) in leukemia patients undergoing HSCT. At TP2, there was a significant improvement in PHQ-9 (<i>p</i> = 0.001), MOCA (<i>p</i> < 0.0001), functional scale (<i>p</i> < 0.0001) and global health & QoL scale (<i>p</i> = 0.001) of EORTC QLQ C30 scores whereas there was a significant decrease in symptom scale of EORTC QLQ C30 score (<i>p</i> = 0.005). Furthermore, at TP2 a statistically significant (<i>p</i> < 0.05) negative correlation was observed between MOCA and symptom scale of EORTC QLQ C30 after Pearson correlation analysis. In conclusion, post-30 days of HSCT there was alleviation in depressive behavior, cognition, and QoL in leukemia patients compared to before therapy.</p><p><strong>Graphical abstract: </strong></p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10831021/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41707076","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Lower Levels of TET2 Gene Expression, with a Higher Level of TET2 Promoter Methylation in Patients with AML; Evidence for the Role of Aberrant Methylation in AML Pathogenesis.","authors":"Bahare Ghasemi, Javad Ahmadi, Farhad Zaker, Tahere Tabatabaei, Masoumeh Kiani-Zadeh, Ahmad Kazemi","doi":"10.1007/s12288-023-01673-y","DOIUrl":"10.1007/s12288-023-01673-y","url":null,"abstract":"<p><p>DNA methylation is a key epigenetic mechanism that is dysregulated in leukemia and plays a significant role in leukemogenesis. Ten-eleven translocation 2 (TET2) is one of the most frequently mutated genes among the DNA methylation regulators in hematologic malignancies, indicating its tumor-suppressor function. In this study, we investigated the expression and methylation status of TET2 in patients with AML. Quantitative RT-PCR was used to evaluate TET2 expression in peripheral blood mononuclear cells (PBMCs) from 51 newly diagnosed AML patients and 50 healthy controls. The methylation-sensitive high-resolution melting (MS-HRM) method was used in 45 patients with AML and 15 healthy controls to evaluate the promoter methylation of TET2. TET2 expression was significantly downregulated (<i>P</i> < 0.0001) in patients with AML compared to that in healthy controls. Furthermore, the methylation level of the TET2 promoter was significantly different between patients and controls. Aberrant methylation of the TET2 promoter was observed in 53.3% of the patients. Interestingly, a negative (- 0.3138) and significant (<i>P</i> = 0.0358) correlation between TET2 methylation and expression was found. The survival of patients with downregulated TET2 was poorer than that of other patients. TET2 gene expression was significantly downregulated while the promoter methylation was higher in patients, indicating that TET2 may be a tumor suppressor gene and a prognostic factor in AML and that transcriptional silencing of the TET2 gene may play a role in AML pathogenesis. Since epigenetic mechanisms are reversible, abnormal TET2 methylation could become a therapeutic target in the future.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10831019/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45482833","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mona ElTagui, Mona El-Ghamrawy, Shareef Karam AlDeeb, Mariam Saad Nassim
{"title":"Metformin as a Fetal Hemoglobin Inducer in Non-transfusion Dependent Thalassemia Patients.","authors":"Mona ElTagui, Mona El-Ghamrawy, Shareef Karam AlDeeb, Mariam Saad Nassim","doi":"10.1007/s12288-023-01662-1","DOIUrl":"10.1007/s12288-023-01662-1","url":null,"abstract":"<p><p>Non-transfusion dependent thalassemia (NTDT) refers to a group of thalassemic disorders who do not need regular transfusions for survival, however it may be needed in certain conditions. Metformin was reported as a potential fetal hemoglobin (HbF) inducing agent in vitro but its efficacy and safety in vivo was not fully studied. This is a prospective interventional study aimed at studying the effect of metformin on HbF change in NTDT.</p><p><strong>Methods: </strong>Patients with established diagnosis of NTDT were enrolled. They were receiving a stable fixed dose of Hydroxyurea over the last 3 months. Patients were divided into two groups: a group that received Metformin for 6 months (Metformin group) and a control group. Complete blood picture, reticulocytic count, hemoglobin electrophoresis, liver enzymes, bilirubin, kidney functions, LDH and random blood sugar were performed at onset, 3 and 6 months of the study. All adverse events were recorded.</p><p><strong>Results: </strong>Forty two patients aged 12-23 years were enrolled. Metformin intake over 6 months did not show any statistically significant difference in clinical or the laboratory variables of efficacy when compared to the control group apart from reticulocytic count which was higher in Metformin group throughout the study.</p><p><strong>Conclusion: </strong>Metformin intake, in addition to hydroxyurea, did not yield any extra benefit among patients with NTDT.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10831011/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42434959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Can Alp Genç, Dilek Gürlek Gökçebay, Vildan Koşan Çulha, Zühre Kaya, Namık Yaşar Özbek
{"title":"Comparison Pharmacokinetic Dosing Tools in Hemophilia A Children.","authors":"Can Alp Genç, Dilek Gürlek Gökçebay, Vildan Koşan Çulha, Zühre Kaya, Namık Yaşar Özbek","doi":"10.1007/s12288-023-01671-0","DOIUrl":"10.1007/s12288-023-01671-0","url":null,"abstract":"<p><p>Prophylaxis is the gold standard for the management of hemophilia A patients. It has been shown that prophylaxis regulated with pharmacokinetic (PK) data reduces frequency of bleeding and cost of treatment. To determine the best prophylaxis regimen, PK dosing tools using the Bayesian method have been developed. We aimed to compare two PK dosing tools. Blood samples were drawn before, 4, 24, and 48 h after FVIII infusions from patients with severe hemophilia A and inhibitor negative. FVIII levels were measured by PTT-based one-stage assay method. PK parameters obtained using WAPPS and myPKFiT, which are web-accessible PK dosing tools using Bayesian algorithm, and daily prophylaxis dose estimated by the programs were compared. Forty-two hemophilia A patients [median age 13 years (IQR 8.9-16.4)] included in the study. There was no difference between the daily dose of FVIII given for prophylaxis and the dose recommended by the myPKFiT for the 1% trough level; whereas, a significant difference was found with the WAPPS. The half-lives of FVIII did not differ between the two dosing tools; however, significant differences were found in the estimated dose, clearances, and times to 1% trough level. There was no significant difference between PK data of patients who received Advate® and those who received non-Advate® factor concentrates. Choice of PK dosing tool can affect recommended FVIII dose. However, target trough levels should be individualized according to bleeding phenotype and daily activity of patient.</p><p><strong>Supplementary information: </strong>The online version contains supplementary material available at 10.1007/s12288-023-01671-0.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10830962/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46870539","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Prevalence of Pulmonary Arterial Hypertension in Patients with Beta Thalassemia Major.","authors":"Masoumeh Kahnoji, Saeid Bitaraf, Narjes Soltani, Hamidreza Esmaeili-Nadimi","doi":"10.1007/s12288-023-01678-7","DOIUrl":"10.1007/s12288-023-01678-7","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) remains a concern in patients with Beta thalassemia major (TM). However, this study aims to investigate the prevalence of PAH in TM patients in Kerman City placed in the south of Iran. In this cross-sectional study on 271 IranianBeta-thalassemia patients (mean age 19.8 <math><mo>±</mo></math> 7.9 in the range 10-60), Age, sex, weekly deferral taking, the number of blood transfusion in a month, and last echocardiography report data was gathered from every patient's file. SPSS for Windows (version 21) was used for analysis. According to the results, the prevalence of PAH was 10.3% (28 patients). PAH had significant associations with weekly deferral consumption (<i>P</i> = 0.028), ferritin serum level (<i>P</i> = 0.001), and ejection fraction (EF) (<i>P</i> = 0.001). PAH is positively associated with deferral consumption and is negatively associated with EF (respectively, <i>P</i> = 0.031, <i>P</i> = 0.001). It is very important to consider prevention and treatment for decreasing the mortality and morbidity of PAH patients in health planning and policy.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10831030/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49093875","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"B12 Deficiency is the Commonest Cause of Anaemia During Pregnancy in Northern India: Study from a Tertiary Care Institute.","authors":"Ankita Bansal, Vanita Suri, Pooja Sikka, Savita Verma Attri, Neelam Varma, Shiv Sajan Saini, Akshaya Goyal, Pankaj Malhotra","doi":"10.1007/s12288-023-01682-x","DOIUrl":"10.1007/s12288-023-01682-x","url":null,"abstract":"<p><p>Iron deficiency anemia is considered the leading cause of anemia during pregnancy; however, there is a lack of comprehensive studies on the etiological factors of anemia in pregnant women. The objective of this study was to systematically investigate the causes of anemia in pregnancy. Five hundred women with hemoglobin levels < 11 g/dl between 6 and 40 weeks of pregnancy underwent a complete hemogram, iron studies, serum folate, serum B12, serum copper, and serum zinc level assessments using standard methods. The median age of the patients was 26 years (range 24-29 years). The majority of patients were in the third trimester (449/500, 89.8%). Among the patients, 325 (65%) had vitamin B12 deficiency, with 159 (31.8%) having isolated B12 deficiency and 142 (28.4%) having combined B12 and iron deficiency. Isolated iron deficiency anemia was present in 74 patients (14.8%). Additionally, 28 patients (5.6%) had beta-thalassemia minor, and anemia of chronic disease was found in 17.2% (86) of the patients. Vitamin B12 deficiency was the most common cause of anemia, followed by combined B12 and iron deficiency. Further studies in diverse populations are warranted as they have broader implications for nutrient supplementation during pregnancy.</p><p><strong>Supplementary information: </strong>The online version contains supplementary material available at 10.1007/s12288-023-01682-x.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10830963/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47809290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}