Blood Cells Molecules and Diseases最新文献

Corrigendum to “Clinical utility of relative telomere length analysis in pediatric bone marrow failure” [Blood Cells Mol. Dis. 109 (2024) 102882] 更正："相对端粒长度分析在小儿骨髓衰竭中的临床应用" [Blood Cells Mol. Dis. 109 (2024) 102882]。

IF 2.1 4区医学

Blood Cells Molecules and Diseases Pub Date : 2024-11-07 DOI: 10.1016/j.bcmd.2024.102899

Shilpa Amatya , Prateek Bhatia , Sudhanshi Raina , Sreejesh Sreedharanunni , Minu Singh , Emine Rahman , M.V. Archana , Amita Trehan

引用次数: 0

Marked microcytosis and increased transferrin saturation: Think about variants in SLC11A2 (DMT1) 明显的小红细胞症和转铁蛋白饱和度升高：考虑 SLC11A2（DMT1）的变异。

IF 2.1 4区医学

Blood Cells Molecules and Diseases Pub Date : 2024-11-02 DOI: 10.1016/j.bcmd.2024.102898

Alexandre Raynor , Katell Peoc'h , Camille Boi , Hana Manceau , Serge Pissard , Karim Diallo , Caroline Kannengiesser , Pierre Rohrlich

引用次数: 0

Identification of Nfel1a and Nfel3 as novel regulators for zebrafish thrombopoiesis 鉴定 Nfel1a 和 Nfel3 作为斑马鱼血栓形成的新型调节器

IF 2.1 4区医学

Blood Cells Molecules and Diseases Pub Date : 2024-10-10 DOI: 10.1016/j.bcmd.2024.102897

Weam Fallatah, Sanchi Dhinoja, Ayah Al Qaryoute, Jabila Mary, Vallerie Cheng, Pudur Jagadeeswaran

{"title":"Identification of Nfel1a and Nfel3 as novel regulators for zebrafish thrombopoiesis","authors":"Weam Fallatah, Sanchi Dhinoja, Ayah Al Qaryoute, Jabila Mary, Vallerie Cheng, Pudur Jagadeeswaran","doi":"10.1016/j.bcmd.2024.102897","DOIUrl":"10.1016/j.bcmd.2024.102897","url":null,"abstract":"<div><div>In mammalian hematopoiesis, megakaryocytes mature and become polyploid in the bone marrow before releasing platelets into circulation. In contrast, fish produce thrombocytes in kidney marrow, where young thrombocytes undergo maturation in circulation, akin to platelets. Despite morphological differences, our single-cell sequencing revealed significant gene expression similarities between fish thrombocytes and mammalian megakaryocytes, including Nfe2, which is crucial in platelet production. In addition to <em>nfe2</em> expression, we found four <em>nfe2-</em>related genes, <em>nfe2I1a, nfe2I1b, nfe2I2a,</em> and <em>nfe2I3,</em> were expressed in mature thrombocytes. However, only <em>nfe2, nfe2l2a,</em> and <em>nfe2l3</em> are expressed in young thrombocytes. Thus, we hypothesized that Nfe2-related factors may also be involved in thrombocyte production. To address this, we knocked down the four novel <em>nfe2-</em>related genes by the piggyback method and found both young and mature thrombocytes reduced when <em>nfe2, nfe2l1a,</em> and <em>nfe2l3</em> were knocked down. They also exhibited greater gill bleeding and prolonged time to occlusion (TTO) compared to controls. In summary, our study shows Nfe2I1a and Nfe2l3 as novel transcription factors that are positive regulators influencing adult zebrafish thrombopoiesis.</div></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"110 ","pages":"Article 102897"},"PeriodicalIF":2.1,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142442845","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

Hemophagocytic lymphohistiocytosis associated with immune checkpoint inhibitor use: A review of the current knowledge and future directions 与使用免疫检查点抑制剂相关的嗜血细胞淋巴组织细胞增多症：回顾当前知识和未来方向。

IF 2.1 4区医学

Blood Cells Molecules and Diseases Pub Date : 2024-09-30 DOI: 10.1016/j.bcmd.2024.102896

Charlotte S. Walmsley , Zachary Schoepflin , Charlotte De Brabandt , Deepa Rangachari , Shana Berwick , Rushad Patell

{"title":"Hemophagocytic lymphohistiocytosis associated with immune checkpoint inhibitor use: A review of the current knowledge and future directions","authors":"Charlotte S. Walmsley , Zachary Schoepflin , Charlotte De Brabandt , Deepa Rangachari , Shana Berwick , Rushad Patell","doi":"10.1016/j.bcmd.2024.102896","DOIUrl":"10.1016/j.bcmd.2024.102896","url":null,"abstract":"<div><div>Hemophagocytic lymphohistiocytosis (HLH) is a severe and often lethal inflammatory syndrome characterized by excessive immune activation leading to fever, cytopenias, and multiorgan involvement. Immune checkpoint inhibitors (ICIs) are central to many contemporary cancer regimens, but their use is associated with immune-related adverse events. Here, we report a case of ICI-induced HLH successfully treated with single agent dexamethasone and provide a scoping review of the literature for cases of ICI-induced HLH with a focus on treatment strategies and outcomes. Using the Medline database, we searched for cases of ICI-associated HLH, with a total of 51 cases reported between 2017 and 2023. Our results underscore the severe nature of this disease, with a 13.7 % mortality rate across 51 case reports. Treatment strategies for ICI-induced HLH were variable: steroids alone (56.9 %), steroids with etoposide (17.6 %), steroids with tociluzumab (11.8 %), among other combinations. Our literature review indicates that steroids alone may be sufficient treatment in some cases of ICI-HLH, with comparable mortality with steroids alone (<em>n</em> = 29) (13.8 %) to that of cases treated with both steroids and immunomodulators (<em>n</em> = 15, 13.3 %). Moreover, all patients treated with steroids and tocilizumab survived (<em>n</em> = 6), suggesting that tocilizumab may be a reasonable next line of therapy when steroid monotherapy proves inadequate. We propose an outline for investigation and treatment of this rare complication of ICI use. Finally, we discuss possible future approaches to develop evidence-based strategies for the diagnosis and management of ICI-induced HLH including the importance of integrating the role of patient community involvement.</div></div>","PeriodicalId":8972,"journal":{"name":"Blood Cells Molecules and Diseases","volume":"110 ","pages":"Article 102896"},"PeriodicalIF":2.1,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142375048","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

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, Aranxa Torres-Caballero, 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 >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 , Mei Yokoyama , Hiromi Hano , Aoi Morita , Midori Matsushita , Tetsuya Tagami , 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 , Ayesha Butt , Vincent P. Schulz , Henry M. Rinder , Alfred I. Lee , Patrick G. Gallagher , John Hwa , 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

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, 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","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

引用次数: 0