Blood Cells Molecules and Diseases最新文献

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Further biological characterization of small molecules UM171 and SR1: In vitro effects on three hematopoietic cell populations from human cord blood 小分子 UM171 和 SR1 的进一步生物学特征:对人类脐带血中三种造血细胞群的体外效应。
IF 2.1 4区 医学
Blood Cells Molecules and Diseases Pub Date : 2024-09-18 DOI: 10.1016/j.bcmd.2024.102895
Patricia Flores-Guzman, Aranxa Torres-Caballero, Hector Mayani
{"title":"Further biological characterization of small molecules UM171 and SR1: In vitro effects on three hematopoietic cell populations from human cord blood","authors":"Patricia Flores-Guzman,&nbsp;Aranxa Torres-Caballero,&nbsp;Hector Mayani","doi":"10.1016/j.bcmd.2024.102895","DOIUrl":"10.1016/j.bcmd.2024.102895","url":null,"abstract":"<div><div>Small molecules UM171 and SR1 have already been taken into clinically-oriented protocols for the ex vivo expansion of hematopoietic stem (HSCs) and progenitor (HPCs) cells. In order to gain further insight into their biology, in the present study we have assessed their effects, both individually and in combination, on the in vitro long-term proliferation and expansion of HSCs and HPCs contained within three different cord blood-derived cell populations: MNCs (CD34<sup>+</sup> cells = 0.8 %), LIN<sup>−</sup> cells (CD34<sup>+</sup> cells = 41 %), and CD34<sup>+</sup> cells (CD34<sup>+</sup> cells &gt;98 %). Our results show that when added to cultures in the absence of recombinant stimulatory cytokines, neither molecule had any effect. In contrast, when added in the presence of hematopoietic cytokines, UM171 and SR1 had significant stimulatory effects on cell proliferation and expansion in cultures of LIN<sup>−</sup> and CD34<sup>+</sup> cells. No significant effects were observed in cultures of MNCs. The effects of both molecules were more pronounced in cultures with the highest proportion of CD34<sup>+</sup> cells, and the greatest effects were observed when both molecules were added in combination. In the absence of small molecules, cell numbers reached a peak by days 25–30, and then declined; whereas in the presence of UM171 or/and SR1 cell numbers were sustained up to day 45 of culture. Our results indicate that besides CD34<sup>+</sup> cells, LIN<sup>−</sup> cells could also be used as input cells in clinically-oriented expansion protocols, and that using both molecules simultaneously would be a better approach than using only one of them.</div></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"110 ","pages":"Article 102895"},"PeriodicalIF":2.1,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142280115","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}
引用次数: 0
Growth hormone is involved in GATA1 gene expression via STAT5B in human erythroleukemia and monocytic cell lines 生长激素通过 STAT5B 参与人类红细胞白血病和单核细胞系中 GATA1 基因的表达。
IF 2.1 4区 医学
Blood Cells Molecules and Diseases Pub Date : 2024-09-18 DOI: 10.1016/j.bcmd.2024.102894
Mana Mitsutani , Mei Yokoyama , Hiromi Hano , Aoi Morita , Midori Matsushita , Tetsuya Tagami , Kenji Moriyama
{"title":"Growth hormone is involved in GATA1 gene expression via STAT5B in human erythroleukemia and monocytic cell lines","authors":"Mana Mitsutani ,&nbsp;Mei Yokoyama ,&nbsp;Hiromi Hano ,&nbsp;Aoi Morita ,&nbsp;Midori Matsushita ,&nbsp;Tetsuya Tagami ,&nbsp;Kenji Moriyama","doi":"10.1016/j.bcmd.2024.102894","DOIUrl":"10.1016/j.bcmd.2024.102894","url":null,"abstract":"<div><div>GATAs are a family of transcription factors consisting of six members. Particularly, GATA1 and GATA2 have been reported to promote the development of erythrocytes, megakaryocytes, eosinophils, and mast cells. However, little information is available on the extracellular ligands that promote GATA1 expression. We evaluated whether growth hormone (GH) is an extracellular stimulator that participates in the signal transduction of GATAs, focusing on GATA1 expression in hematopoietic cell lineages. We used a reporter assay, RT-PCR, real-time quantitative PCR, and western blotting to evaluate GH-induced expression of GATA1 and GATA2 in the human erythroleukemic cell line K562 and the non-erythroid cell line U937. GATA1 expression in these hematopoietic cell lines increased at the transcriptional and protein levels in the presence of GH, and was inhibited by a STAT5 specific inhibitor. Cells transfected with activated STAT5B showed increased expression of GATA1. We identified functional STAT5B consensus sequences as binding site-158 bp from the transcription starting site in the GATA1 promoter region. These results suggest that GH directly induces GATA1 expression via GHR/JAK/STAT5 and is related to hematopoietic cell proliferation.</div></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"110 ","pages":"Article 102894"},"PeriodicalIF":2.1,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142280116","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}
引用次数: 0
Phenotypic and genotypic evaluation of bleeding diagnostic dilemmas: Two case studies 出血诊断难题的表型和基因型评估:两个案例研究
IF 2.1 4区 医学
Blood Cells Molecules and Diseases Pub Date : 2024-09-10 DOI: 10.1016/j.bcmd.2024.102893
Sean X. Gu , Ayesha Butt , Vincent P. Schulz , Henry M. Rinder , Alfred I. Lee , Patrick G. Gallagher , John Hwa , Robert D. Bona
{"title":"Phenotypic and genotypic evaluation of bleeding diagnostic dilemmas: Two case studies","authors":"Sean X. Gu ,&nbsp;Ayesha Butt ,&nbsp;Vincent P. Schulz ,&nbsp;Henry M. Rinder ,&nbsp;Alfred I. Lee ,&nbsp;Patrick G. Gallagher ,&nbsp;John Hwa ,&nbsp;Robert D. Bona","doi":"10.1016/j.bcmd.2024.102893","DOIUrl":"10.1016/j.bcmd.2024.102893","url":null,"abstract":"<div><p>Inherited platelet disorders (IPDs) are a heterogeneous group of conditions that present significant challenges in diagnosis and management. Here, we report two cases of patients presenting with clinically significant bleeding but with unclear etiologies by conventional clinical laboratory testing. Further evaluation, utilizing a combination of high-dimensional multiplexed mass cytometry and genetic sequencing, revealed the underlying causes of bleeding in both cases, leading to definitive diagnoses. These cases underscore the potential utility of combined multimodal approaches in evaluating patients with bleeding disorders. Moreover, these high-parameter methods can offer substantial mechanistic insights and can enhance our understanding of the molecular pathogenesis of IPDs. Future studies involving larger patient cohorts are needed to further validate this strategy, directly comparing its diagnostic yield and accuracy with current clinical laboratory testing approaches, which can ultimately improve patient care.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"110 ","pages":"Article 102893"},"PeriodicalIF":2.1,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142163317","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}
引用次数: 0
Corrigendum to “Outpatient ATG-free hematopoietic transplantation for severe aplastic anemia in limited-resource environments offers excellent results: Data from a single LATAM center” [Blood Cells, Mol. Dis. 109 (2024) 102885] 更正:"在资源有限的环境中,门诊无 ATG 造血移植治疗重型再生障碍性贫血效果极佳:来自一个拉美医学中心的数据" [Blood Cells, Mol. Dis. 109 (2024) 102885]。
IF 2.1 4区 医学
Blood Cells Molecules and Diseases Pub Date : 2024-09-06 DOI: 10.1016/j.bcmd.2024.102886
José Carlos Jaime-Pérez, Mariana González-Treviño, Andrés Gómez-De León, Miguel A. Campos-Bocardo, Renata V. Barragán-Longoria, Olga Graciela Cantú-Rodríguez, César Homero Gutiérrez-Aguirre, David Gómez-Almaguer
{"title":"Corrigendum to “Outpatient ATG-free hematopoietic transplantation for severe aplastic anemia in limited-resource environments offers excellent results: Data from a single LATAM center” [Blood Cells, Mol. Dis. 109 (2024) 102885]","authors":"José Carlos Jaime-Pérez,&nbsp;Mariana González-Treviño,&nbsp;Andrés Gómez-De León,&nbsp;Miguel A. Campos-Bocardo,&nbsp;Renata V. Barragán-Longoria,&nbsp;Olga Graciela Cantú-Rodríguez,&nbsp;César Homero Gutiérrez-Aguirre,&nbsp;David Gómez-Almaguer","doi":"10.1016/j.bcmd.2024.102886","DOIUrl":"10.1016/j.bcmd.2024.102886","url":null,"abstract":"","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"110 ","pages":"Article 102886"},"PeriodicalIF":2.1,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1079979624000640/pdfft?md5=52e69c13f2e3990cdf5b98e200a3ecce&pid=1-s2.0-S1079979624000640-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142145049","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}
引用次数: 0
Outpatient ATG-free hematopoietic transplantation for aplastic anemia in limited-resource environments offers excellent results: Data from a single LATAM center 在资源有限的环境中,门诊无ATG造血移植治疗再生障碍性贫血效果极佳:来自拉丁美洲和加勒比海地区一家中心的数据
IF 2.1 4区 医学
Blood Cells Molecules and Diseases Pub Date : 2024-08-20 DOI: 10.1016/j.bcmd.2024.102885
José Carlos Jaime-Pérez, Mariana González-Treviño, Andrés Gómez-De León, Miguel A. Campos-Bocardo, Renata V. Barragán-Longoria, Olga Graciela Cantú-Rodríguez, César Homero Gutiérrez-Aguirre, David Gómez-Almaguer
{"title":"Outpatient ATG-free hematopoietic transplantation for aplastic anemia in limited-resource environments offers excellent results: Data from a single LATAM center","authors":"José Carlos Jaime-Pérez,&nbsp;Mariana González-Treviño,&nbsp;Andrés Gómez-De León,&nbsp;Miguel A. Campos-Bocardo,&nbsp;Renata V. Barragán-Longoria,&nbsp;Olga Graciela Cantú-Rodríguez,&nbsp;César Homero Gutiérrez-Aguirre,&nbsp;David Gómez-Almaguer","doi":"10.1016/j.bcmd.2024.102885","DOIUrl":"10.1016/j.bcmd.2024.102885","url":null,"abstract":"<div><h3>Objective</h3><p>To document the results of outpatient hematopoietic stem cell transplantation (HSCT) from the peripheral blood (PB) of sibling donors without anti-thymocyte globulin (ATG) in the conditioning regimen.</p></div><div><h3>Material and methods</h3><p>Patients from a low-income population with severe AA who received a PB, unmanipulated sibling HLA-identical HSCT between 2000 and 2020 at a single institution were studied. Survival was the primary outcome.</p></div><div><h3>Results</h3><p>Forty-one transplants were performed. Time between diagnosis and transplant was five months (1–104). Median age was 37 (range, 4–61) years; 25 (61 %) recipients were males and 32 (78 %) had treatment failure, 9 (22 %) have not received treatment. ATG was administered in 5 (12.2 %) cases; the graft source was PB in 38 (92.7 %) transplants. Twenty-six (63.4 %) transplants were carried out in the outpatient setting. Infections developed in 14 (34.1 %) patients. Primary graft failure (GF) occurred in 3 (7.3 %) patients. The 15-year OS was 81 %, EFS was 77.4 %. Patients with high pre-HSCT transfusion burden had lower OS (<em>p</em> = 0.035) and EFS (<em>p</em> = 0.026). Previous treatment failure and age were not associated with lower OS (<em>p</em> = 0.115, <em>p</em> = 0.069) or EFS (<em>p</em> = 0.088, <em>p</em> = 0.5, respectively).</p></div><div><h3>Conclusions</h3><p>HLA-identical T-cell replete outpatient HSCT from the PB of sibling donors for AA patients using ATG-free conditioning offers excellent long-term survival.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"109 ","pages":"Article 102885"},"PeriodicalIF":2.1,"publicationDate":"2024-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142049734","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}
引用次数: 0
Red cell distribution width as a bellwether of prognosis 红细胞分布宽度是预后的风向标
IF 2.1 4区 医学
Blood Cells Molecules and Diseases Pub Date : 2024-08-14 DOI: 10.1016/j.bcmd.2024.102884
Marshall A. Lichtman
{"title":"Red cell distribution width as a bellwether of prognosis","authors":"Marshall A. Lichtman","doi":"10.1016/j.bcmd.2024.102884","DOIUrl":"10.1016/j.bcmd.2024.102884","url":null,"abstract":"<div><p>The red cell distribution width (RDW) is a standard variable reported in the complete blood count. It has been found to have a consistent relationship to life expectancy in older individuals, prognosis in patients with cardiovascular disease, outcome in those with hematological and non-hematological neoplasms and in a variety of medical circumstances such as non-cardiovascular or cancer related critical illness and postoperative outcome from various procedures. This report reviews some of the key medical publications establishing these relationships with RDW. The precise pathobiological processes that explain the predictive value of the RDW in this wide array of circumstances or why an alteration in erythropoiesis (exaggerated red cell size variation) occurs is uncertain. The possible role of inflammation has been one hypothesis considered, but not established.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"109 ","pages":"Article 102884"},"PeriodicalIF":2.1,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142012225","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}
引用次数: 0
Clinical characteristics, laboratory features and genetic profile of hemoglobin E (HBB:c.79 G > A)/β (nucleotide -28 A > G) (HBB:c.-78 A > G) -thalassemia subjects identified from community- and hospital-recruited cohorts 从社区和医院招募的队列中确定的血红蛋白 E (HBB:c.79 G > A)/β (核苷酸 -28 A > G) (HBB:c.-78 A > G) - 地中海贫血症受试者的临床特征、实验室特征和遗传特征
IF 2.1 4区 医学
Blood Cells Molecules and Diseases Pub Date : 2024-08-08 DOI: 10.1016/j.bcmd.2024.102883
Piyatida Chumnumsiriwath, Prissana Charoenporn, Sawichayaporn Jermnim, Pawanrat Suannum, Monthira Samaisombat, Akamon Tapprom, Rawisut Deoisares, Peerapon Wong
{"title":"Clinical characteristics, laboratory features and genetic profile of hemoglobin E (HBB:c.79 G > A)/β (nucleotide -28 A > G) (HBB:c.-78 A > G) -thalassemia subjects identified from community- and hospital-recruited cohorts","authors":"Piyatida Chumnumsiriwath,&nbsp;Prissana Charoenporn,&nbsp;Sawichayaporn Jermnim,&nbsp;Pawanrat Suannum,&nbsp;Monthira Samaisombat,&nbsp;Akamon Tapprom,&nbsp;Rawisut Deoisares,&nbsp;Peerapon Wong","doi":"10.1016/j.bcmd.2024.102883","DOIUrl":"10.1016/j.bcmd.2024.102883","url":null,"abstract":"<div><p>Despite several existing laboratory-based studies of hemoglobin (Hb) E (HBB:c.79 G &gt; A)/ β (nucleotide (NT) -28 A &gt; G) (HBB:c.-78 A &gt; G) -thalassemia, no reports have ever provided clinical severity information as well as dependency of blood transfusion. Previously, a comparative study of community- and hospital-recruited Hb E/β-thalassemia subjects was conducted in the lower northern Thailand between June 2020 and December 2021. A mobile medical team visited each community hospital on-site, collecting clinical severity parameters, and conducting Hb and DNA analyses. The control included Hb E/β-thalassemia patients undergoing transfusions. Subgroup study of adult Hb E/β (NT -28 A &gt; G) -thalassemia subjects was subsequently conducted. Additional pediatric individuals were recruited from prenatal diagnosis databases. Twenty adult and nine pediatric subjects were enrolled; all were classified as having mild disease severity. Twenty-two individuals (75.9 %) were asymptomatic. Six adults (20.7 %) required blood transfusion. The mean Hb level of subjects without transfusion (23 [79.3 %]) was 10.77 ± 1.10 g/dL. Hb analysis revealed a distinct EFA pattern with low Hb F fraction. The positive impact of genetic modifiers could not be statistically demonstrated except rs7482144-<em>Xmn</em>I. These findings could provide essential information for parents carrying fetuses with Hb E/β (NT -28 A &gt; G) -thalassemia.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"109 ","pages":"Article 102883"},"PeriodicalIF":2.1,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141997700","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}
引用次数: 0
Erythrocyte deformability correlates with systemic inflammation 红细胞变形性与全身炎症相关
IF 2.1 4区 医学
Blood Cells Molecules and Diseases Pub Date : 2024-08-02 DOI: 10.1016/j.bcmd.2024.102881
Carmen Jacob , Lakeesha Piyasundara , Maria Bonello , Michael Nathan , Stefania Kaninia , Aravinthan Varatharaj , Noémi Roy , Ian Galea
{"title":"Erythrocyte deformability correlates with systemic inflammation","authors":"Carmen Jacob ,&nbsp;Lakeesha Piyasundara ,&nbsp;Maria Bonello ,&nbsp;Michael Nathan ,&nbsp;Stefania Kaninia ,&nbsp;Aravinthan Varatharaj ,&nbsp;Noémi Roy ,&nbsp;Ian Galea","doi":"10.1016/j.bcmd.2024.102881","DOIUrl":"10.1016/j.bcmd.2024.102881","url":null,"abstract":"<div><p>Recent evidence suggests that systemic conditions, particularly those associated with inflammation, can affect erythrocyte deformability in the absence of haematological conditions. In this exploratory study, we investigated the relationship between systemic inflammatory status and erythrocyte deformability (using osmotic gradient ektacytometry) in a heterogenous study population consisting of individuals with no medical concerns, chronic conditions, and acute illness, providing a wide range of systemic inflammation severity.</p><p>22 participants were included in a prospective observational study. Maximum Elongation Index (EI<sub>max</sub>) in ektacytometry served as the readout for erythrocyte deformability. Inflammatory status was assessed using C-reactive protein (CRP) and self-reported symptoms associated with inflammatory activation (Sickness Questionnaire Scores, SicknessQ).</p><p>In a univariate linear regression, both CRP and SicknessQ scores significantly predicted EI<sub>max</sub> (CRP: F(1,20) = 7.751, <em>p</em> &lt; 0.05 (0.011), R<sup>2</sup> = 0.279; SicknessQ: F(1,18) = 4.831, p &lt; 0.05 (0.041), R<sup>2</sup> = 0.212). Sensitivity analyses with multivariable linear regression correcting for age showed concordant findings.</p><p>Results suggest a linear relationship between erythrocyte deformability and biochemical and clinical markers of systemic inflammation. Replication of findings in a larger study, and mechanisms and clinical consequences need further in investigation.</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"109 ","pages":"Article 102881"},"PeriodicalIF":2.1,"publicationDate":"2024-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1079979624000597/pdfft?md5=f23be722aa973fc8705021ee36b6de01&pid=1-s2.0-S1079979624000597-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141991059","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}
引用次数: 0
Clinical utility of relative telomere length analysis in pediatric bone marrow failure 相对端粒长度分析在小儿骨髓衰竭中的临床应用。
IF 2.1 4区 医学
Blood Cells Molecules and Diseases Pub Date : 2024-07-31 DOI: 10.1016/j.bcmd.2024.102882
Shilpa Amatya , Prateek Bhatia , Sudhanshi Raina , Sreejesh Sreedharanunni , Minu Singh , Emine Rahman , M.V. Archana , Amita Trehan
{"title":"Clinical utility of relative telomere length analysis in pediatric bone marrow failure","authors":"Shilpa Amatya ,&nbsp;Prateek Bhatia ,&nbsp;Sudhanshi Raina ,&nbsp;Sreejesh Sreedharanunni ,&nbsp;Minu Singh ,&nbsp;Emine Rahman ,&nbsp;M.V. Archana ,&nbsp;Amita Trehan","doi":"10.1016/j.bcmd.2024.102882","DOIUrl":"10.1016/j.bcmd.2024.102882","url":null,"abstract":"<div><h3>Introduction</h3><p>Telomere length related studies are limited in pediatric marrow failure cases due to difficulty in establishing population specific age related normograms. Moreover, there is paucity of data related to clinical relevance of telomere length in idiopathic aplastic anemia (IAA) and non telomere biology inherited bone marrow failure syndrome (IBMFS) cases. Methodology: Hence, in current study we investigated Relative telomere length (RTL) by RQ-PCR in 83 samples as: healthy controls (<em>n</em> = 44), IAA (<em>n</em> = 15) and IBMFS (<em>n</em> = 24). In addition, we performed chromosomal breakage studies and targeted NGS to screen for pathogenic variants. Results &amp; Conclusion: Median RTL was significantly different between control vs. IBMFS (<em>p</em>-0.002), IAA vs. IBMFS (<em>p</em>-0.0075) and DC vs. non-DC IBMFS (<em>p</em>-0.011) but not between control vs. IAA (<em>p</em>-0.46). RTL analysis had clinical utility in differentiating BMF cases as 75 % (9/12) of DC had short/very short telomeres compared to only 17 % (2/12) of non-DC IBMFS, 7 % (1/15) of IAA and 7 % (3/44) of controls (<em>p</em> &lt; 0.001).</p></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"109 ","pages":"Article 102882"},"PeriodicalIF":2.1,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141888457","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}
引用次数: 0
The 50th anniversary of Blood Cells, Molecules and Diseases, 1975–2024 血细胞、分子和疾病》50 周年纪念,1975-2024 年
IF 2.1 4区 医学
Blood Cells Molecules and Diseases Pub Date : 2024-07-19 DOI: 10.1016/j.bcmd.2024.102854
Marshall A. Lichtman
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