Paul Muteb Boma , Stéphanie Luntadila Ngimbi , Junior Makiese Kindundu , Jean Israël Wela , Nathalie Lukanke Ngoie , Valentin Mukeba Ngwamah , Sandra Mbuyi Tshiswaka , Joséphine Kalenga Monga , Jules Mulefu Panda , Bruno Bonnechère
{"title":"Unveiling mortality risk factors in paediatric sickle cell disease patients during acute crises in the Democratic Republic of the Congo","authors":"Paul Muteb Boma , Stéphanie Luntadila Ngimbi , Junior Makiese Kindundu , Jean Israël Wela , Nathalie Lukanke Ngoie , Valentin Mukeba Ngwamah , Sandra Mbuyi Tshiswaka , Joséphine Kalenga Monga , Jules Mulefu Panda , Bruno Bonnechère","doi":"10.1016/j.bcmd.2024.102828","DOIUrl":"10.1016/j.bcmd.2024.102828","url":null,"abstract":"<div><p><span><span>Sickle cell disease (SCD) is a significant health burden in the Democratic Republic of the Congo (DRC). This study aims to identify </span>predictive factors of mortality in SCD children admitted to emergency care in Lubumbashi, DRC. We performed a non-interventional cohort follow-up on SCD patients aged 0 to 16 admitted for a “true emergency”. Demographic, clinical, and biological data were collected. Univariate and multivariate </span>logistic regression<span> analyses were performed to identify significant risk factors associated with mortality. Among the 121 patients included, 24 died during the follow-up period. Univariate regression revealed age, Mikobi score, referral origin, stroke, and severe infection as significant risk factors. Multivariate analyses<span><span><span> identified Hb, WBC, SR, and </span>LDH as predictive factors of mortality. Notably, patients aged 12 to 16 years faced a higher risk, shifting the age of mortality from early to late childhood and adolescence. This study provides valuable insights into mortality risk factors for </span>paediatric SCD patients during acute crises. Early diagnosis, regular follow-up, and therapeutic education are essential to improve patient outcomes and survival rates. These findings contribute to better disease management and targeted interventions, aiming to reduce mortality associated with SCD.</span></span></p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"105 ","pages":"Article 102828"},"PeriodicalIF":2.3,"publicationDate":"2024-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139508528","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}
Avital Mendelson , Yunfeng Liu , Weili Bao , Patricia A. Shi
{"title":"Effect of voxelotor on murine bone marrow and peripheral blood with hematopoietic progenitor cell mobilization for gene therapy of sickle cell disease","authors":"Avital Mendelson , Yunfeng Liu , Weili Bao , Patricia A. Shi","doi":"10.1016/j.bcmd.2024.102824","DOIUrl":"10.1016/j.bcmd.2024.102824","url":null,"abstract":"<div><p>In preparation for hematopoietic stem cell mobilization and collection, current ex vivo gene therapy protocols for sickle cell disease require patients to undergo several months of chronic red cell transfusion. For health care equity, alternatives to red cell transfusion should be available. We examined whether treatment with GBT1118, the murine analog of voxelotor, could be a safe and feasible alternative to red cell transfusion. We found that 3 weeks of treatment with GBT1118 increased the percentage of bone marrow hematopoietic stem cells and upon plerixafor mobilization, the percentage of peripheral blood hematopoietic stem cells. Our data suggest that voxelotor should be further explored for its potential safety and utility as preparation for hematopoietic stem cell mobilization and collection.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"105 ","pages":"Article 102824"},"PeriodicalIF":2.3,"publicationDate":"2024-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1079979624000020/pdfft?md5=b353839cb736711f91015e06fb67f8ca&pid=1-s2.0-S1079979624000020-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139508532","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}
Fernanda D'Avila da Silva , Maria Eduarda de Andrade Galiciolli , Ana Carolina Irioda , Cláudia Sirlene Oliveira , Bruna Candia Piccoli , Alessandro de Souza Prestes , Bruna Cogo Borin , Andre Passaglia Schuch , Estael Ochoa-Rodríguez , Yanier Nuñez-Figueredo , João Batista Teixeira da Rocha
{"title":"Investigation of the cytotoxicity, genotoxicity and antioxidant prospects of JM-20 on human blood cells: A multi-target compound with potential therapeutic applications","authors":"Fernanda D'Avila da Silva , Maria Eduarda de Andrade Galiciolli , Ana Carolina Irioda , Cláudia Sirlene Oliveira , Bruna Candia Piccoli , Alessandro de Souza Prestes , Bruna Cogo Borin , Andre Passaglia Schuch , Estael Ochoa-Rodríguez , Yanier Nuñez-Figueredo , João Batista Teixeira da Rocha","doi":"10.1016/j.bcmd.2024.102827","DOIUrl":"10.1016/j.bcmd.2024.102827","url":null,"abstract":"<div><p><span><span>JM-20 is a 1,5-benzodiazepine compound fused to a dihydropyridine<span><span> fraction with different pharmacological properties. However, its potential toxic effects on blood cells have not yet been reported. Thus, the present study aimed to investigate, for the first time, the possible cytotoxicity of JM-20 through </span>cell viability<span><span>, cell cycle, morphology changes, reactive species (RS) to DCFH-DA, and lipid peroxidation in human leukocytes, its </span>hemolytic<span> effect on human erythrocytes, and its potential DNA </span></span></span></span>genotoxicity using plasmid DNA </span><em>in vitro</em><span>. Furthermore, the compound's ability to reduce the DPPH radical was also measured. Human blood was obtained from healthy volunteers (30 ± 10 years old), and the leukocytes or erythrocytes were immediately isolated and treated with different concentrations of JM-20. A cytoprotective effect was exhibited by 10 μM JM-20 against 1 mM tert-butyl hydroperoxide (t-but-OOH) in the leukocytes. However, the highest tested concentrations of the compound (20 and 50 μM) changed the morphology and caused a significant decrease in the cell viability of leukocytes (p < 0.05, in comparison with Control). All tested concentrations of JM-20 also resulted in a significant increase in intracellular RS as measured by DCFH-DA in these cells (p < 0.05, in comparison with Control). On the other hand, the results point out a potent antioxidant effect of JM-20, which was similar to the classical antioxidant α-tocopherol. The IC</span><sub>50</sub> value of JM-20 against the lipid peroxidation induced by (FeII) was 1.051 μM ± 0.21, while the IC<sub>50</sub> value of α-tocopherol in this parameter was 1.065 μM ± 0.34. Additionally, 50 and 100 μM JM-20 reduced the DPPH radical in a statistically similar way to the 100 μM α-tocopherol (p < 0.05, in comparison with the control). No significant hemolysis in erythrocytes, no cell cycle changes in leukocytes, and no genotoxic effects in plasmid DNA were induced by JM-20 at any tested concentration. The <span><em>in silico</em></span><span> pharmacokinetic<span> and toxicological properties of JM-20, derivatives, and nifedipine were also studied. Here, our findings demonstrate that JM-20 and its putative metabolites exhibit similar characteristics to nifedipine, and the </span></span><em>in vitro</em> and <em>in silico</em> data support the low toxicity of JM-20 to mammals.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"106 ","pages":"Article 102827"},"PeriodicalIF":2.3,"publicationDate":"2024-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139510183","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":"Regarding γ′ fibrinogen levels as a biomarker of COVID-19 respiratory disease severity","authors":"Sangsang Wang , Diao Yu , Junwu Zhang","doi":"10.1016/j.bcmd.2024.102826","DOIUrl":"10.1016/j.bcmd.2024.102826","url":null,"abstract":"","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"105 ","pages":"Article 102826"},"PeriodicalIF":2.3,"publicationDate":"2024-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139499238","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}
Lucy Z. Kornblith , Bindhya Sadhanandhan , Sreepriya Arun , Rebecca Long , Alicia J. Johnson , Jamie Noll , C.N. Ramchand , John K. Olynyk , David H. Farrell
{"title":"Regarding γ′ fibrinogen levels as a biomarker of COVID-19 respiratory disease severity","authors":"Lucy Z. Kornblith , Bindhya Sadhanandhan , Sreepriya Arun , Rebecca Long , Alicia J. Johnson , Jamie Noll , C.N. Ramchand , John K. Olynyk , David H. Farrell","doi":"10.1016/j.bcmd.2024.102825","DOIUrl":"10.1016/j.bcmd.2024.102825","url":null,"abstract":"","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"105 ","pages":"Article 102825"},"PeriodicalIF":2.3,"publicationDate":"2024-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139566576","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}
Brígida Santos , Catarina Ginete , Elisângela Gonçalves , Mariana Delgadinho , Armandina Miranda , Paula Faustino , Ana Paula Arez , Miguel Brito
{"title":"Characterization of a cohort of Angolan children with sickle cell anemia treated with hydroxyurea","authors":"Brígida Santos , Catarina Ginete , Elisângela Gonçalves , Mariana Delgadinho , Armandina Miranda , Paula Faustino , Ana Paula Arez , Miguel Brito","doi":"10.1016/j.bcmd.2023.102822","DOIUrl":"10.1016/j.bcmd.2023.102822","url":null,"abstract":"<div><h3>Background</h3><p>Sickle Cell Anemia (SCA) is a monogenic disease, although its severity and response to treatment are very heterogeneous.</p></div><div><h3>Objectives</h3><p>This study aims to characterize a cohort of Angolan children with SCA and evaluate their response to hydroxyurea (HU) treatment and the potential side effects and toxicity.</p></div><div><h3>Methods</h3><p>The study enrolled 215 patients between 3 and 12 years old before and after the administration of HU, at a fix dose of 20 mg/kg/day for 12 months.</p></div><div><h3>Results</h3><p>A total of 157 patients started HU medication and 141 of them completed the 12-month treatment. After initiating HU treatment, the frequency of clinical events decreased (transfusions 53.4 %, hospitalizations 47.1 %). The response to HU medication varied among patients, with some experiencing an increase in fetal hemoglobin (HbF) of <5 %. The mean increase in HbF was 11.9 %, ranging from 1.8 % to 31 %. Responders to HU treatment were 57 %, inadequate responders 38.7 % and non-adherent 4.2 %. No clinical side effects related to HU were reported. Hematological toxicities were transient and reversible. Children naïve to HU and with lower HbF reported higher number of hospitalizations caused by malaria infection. During HU treatment, the frequency of malaria episodes did not appear to be affected by HbF levels.</p></div><div><h3>Conclusions</h3><p>the present study provided a valuable contribution to the understanding of the clinical and laboratory profiles of Angolan children with SCA. These findings support the evidence that the implementation of prophylactic measures and treatment with HU is associated with increased survival in children with SCA.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"105 ","pages":"Article 102822"},"PeriodicalIF":2.3,"publicationDate":"2024-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1079979623000992/pdfft?md5=6ac4fc1b23206523b69ea849bc5368bf&pid=1-s2.0-S1079979623000992-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139094036","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}
Usman Ali Shams , Isma Javed , Muhammad Fizan , Aqib Raza Shah , Ghulam Mustafa , Muhammad Zubair , Yehia Massoud , Muhammad Qasim Mehmood , Muhammad Asif Naveed
{"title":"Bio-net dataset: AI-based diagnostic solutions using peripheral blood smear images","authors":"Usman Ali Shams , Isma Javed , Muhammad Fizan , Aqib Raza Shah , Ghulam Mustafa , Muhammad Zubair , Yehia Massoud , Muhammad Qasim Mehmood , Muhammad Asif Naveed","doi":"10.1016/j.bcmd.2024.102823","DOIUrl":"10.1016/j.bcmd.2024.102823","url":null,"abstract":"<div><p><span>Peripheral blood smear examination is one of the basic steps in the evaluation of different </span>blood cells<span>. It is a confirmatory step after an automated complete blood count<span> analysis. Manual microscopy is time-consuming and requires professional laboratory expertise. Therefore, the turn-around time for peripheral smear in a health care<span> center is approximately 3–4 hours. To avoid the traditional method of manual counting under the microscope a computerized automation of peripheral blood smear examination has been adopted, which is a challenging task in medical diagnostics. In recent times, deep learning techniques have overcome the challenges associated with human microscopic evaluation of peripheral smears and this has led to reduced cost and precise diagnosis. However, their application can be significantly improved by the availability of annotated datasets. This study presents a large customized annotated blood cell dataset (named the Bio-Net dataset from healthy individuals) and blood cell detection and counting in the peripheral blood smear images. A mini-version of the dataset for specialized WBC-based image processing tasks is also equipped to classify the healthy and mature WBCs in their respective classes. An object detection algorithm called You Only Look Once (YOLO) with a refashion disposition has been trained on the novel dataset to automatically detect and classify blood cells into RBCs, WBCs, and platelets and compare the results with other publicly available datasets to highlight the versatility. In short the introduction of the Bio-Net dataset and AI-powered detection and counting offers a significant potential for advancement in biomedical research for analyzing and understanding biological data.</span></span></span></p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"105 ","pages":"Article 102823"},"PeriodicalIF":2.3,"publicationDate":"2024-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139396173","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":"Unmasking the morphological alteration of erythrocytes among women suffering from PCOS","authors":"Sutithi Dey , Ipsita Chakraborty , Payel Biswas , Ayantika Paul , Pratip Chakraborty , Rajen Haldar","doi":"10.1016/j.bcmd.2023.102821","DOIUrl":"10.1016/j.bcmd.2023.102821","url":null,"abstract":"<div><p><span><span><span>Dyslipidemia<span> is frequently observed in polycystic ovarian syndrome (PCOS). Changes in plasma </span></span>lipid levels<span><span> potentially alter erythrocyte membrane<span> lipid composition due to lack of inbuilt </span></span>lipid synthesis<span><span> machinery. Therefore, development of morphologically altered erythrocytes in PCOS patients with dyslipidemia is expected. However, this has not been established so far. So, we took this opportunity to explore the morphological alterations among dyslipidemic PCO women. We recruited thirty-five dyslipidemic PCOS women (satisfying Rotterdam criteria, without medication) and twenty-five age-matched healthy controls. Scanning electron microscopy revealed a significant increase in the number of stomatocytes, </span>acanthocytes, and </span></span></span>echinocytes<span> in the PCO group. PCO group showed a considerable decrease in plasma antioxidant levels. Elevated lipid peroxidation, </span></span>protein carbonylation<span><span><span><span>, and decreased free thiol group in erythrocyte membrane in PCOS suggest </span>oxidative degradation of the erythrocyte membrane. Elevated intracellular </span>ROS<span> levels, increased methemoglobin formation, and a decrease in NADPH methemoglobin reductase in PCOS also indicate altered physicochemical property of hemoglobin due to oxidative overload. Additionally, these patients exhibit a rise in erythrocyte membrane cholesterol and </span></span>triglyceride<span>, which promotes the membrane to become less fluidic and less fragile. Thus, these results corroborate a potential role in altering erythrocyte morphology among dyslipidemic PCO women.</span></span></p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"105 ","pages":"Article 102821"},"PeriodicalIF":2.3,"publicationDate":"2024-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139094278","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":"Impact of CD34 positive cell dose in donor graft on the outcomes after haploidentical peripheral blood stem cell transplantation with post-transplant cyclophosphamide – A retrospective single-center study with a Japanese cohort","authors":"Yumiko Maruyama , Hidekazu Nishikii , Naoki Kurita , Tatsuhiro Sakamoto , Keiichiro Hattori , Yasuhito Suehara , Yasuhisa Yokoyama , Takayasu Kato , Naoshi Obara , Mamiko Sakata-Yanagimoto , Shigeru Chiba","doi":"10.1016/j.bcmd.2023.102820","DOIUrl":"10.1016/j.bcmd.2023.102820","url":null,"abstract":"<div><h3>Background</h3><p><span>Haploidentical peripheral blood stem cell transplantation<span> (haplo-PBSCT) with post-transplant cyclophosphamide<span> (PTCy) is an important therapeutic option for patients lacking an HLA-matched donor. However, the significance of CD34</span></span></span><sup>+</sup> cell dose in grafts has not been fully elucidated.</p></div><div><h3>Objective</h3><p>We aimed to explore the impact of CD34<sup>+</sup> cell dose on outcomes after haplo-PBSCT with PTCy.</p></div><div><h3>Study Design</h3><p>We retrospectively investigated 111 consecutive patients who underwent haplo-PBSCT with PTCy or HLA-matched PBSCT from related donors.</p></div><div><h3>Results</h3><p>There were no statistically significant differences in 3-year overall survival (<em>p</em> = 0.559) or progression-free survival (<em>p</em><span> = 0.974) between haplo-PBSCT and matched PBSCT. Delayed neutrophil<span> engraftment and a lower incidence of graft-versus-host disease were observed in haplo-PBSCT. The median dose of CD34</span></span><sup>+</sup> cells was 4.9 × 10<sup>6</sup> /kg in 57 haplo-PBSCT and 4.5 × 10<sup>6</sup> /kg in 54 matched PBSCTs. Importantly, patients who underwent haplo-PBSCT with the administration of CD34<sup>+</sup> cell at a dose of ≥4.0 × 10<sup>6</sup> /kg significantly had improved OS (<em>p</em> = 0.015) and decreased incidence of disease relapse (<em>p</em> = 0.001) without increasing incidence of GVHD.</p></div><div><h3>Conclusion</h3><p>Our data suggest that a higher dose of CD34<sup>+</sup> cells in haplo-PBSCT with PTCy positively impacts the outcomes without an increase of GVHD.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"105 ","pages":"Article 102820"},"PeriodicalIF":2.3,"publicationDate":"2023-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139065475","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":"Measurement of red cell, plasma and blood volume: Essential components of diagnostic and research studies of oxygen transport","authors":"Marshall A. Lichtman , Josef T. Prchal","doi":"10.1016/j.bcmd.2023.102819","DOIUrl":"10.1016/j.bcmd.2023.102819","url":null,"abstract":"","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"105 ","pages":"Article 102819"},"PeriodicalIF":2.3,"publicationDate":"2023-12-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138555924","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}