Current Opinion in Hematology最新文献_第9页

Crosstalk between terminal erythropoiesis and granulopoiesis within their common niche: the erythromyeloblastic island. 红细胞生成末期和粒细胞生成末期在其共同的生态位（红细胞母细胞岛）中相互交织。

IF 3.1 3区医学

Current Opinion in Hematology Pub Date : 2023-07-01 Epub Date: 2023-04-25 DOI: 10.1097/MOH.0000000000000767

Laurel Romano, Katie G Seu, Lionel Blanc, Theodosia A Kalfa

{"title":"Crosstalk between terminal erythropoiesis and granulopoiesis within their common niche: the erythromyeloblastic island.","authors":"Laurel Romano, Katie G Seu, Lionel Blanc, Theodosia A Kalfa","doi":"10.1097/MOH.0000000000000767","DOIUrl":"10.1097/MOH.0000000000000767","url":null,"abstract":"Purpose of review: The identity of the erythroblastic island (EBI) macrophage (Mϕ) has been under investigation for decades since it was recognized as the first hematopoietic niche 'nursing' terminal erythropoiesis. This review will focus on the current insights to the characteristics and the role of the EBI Mϕ balancing terminal erythropoiesis and granulopoiesis.Recent findings: While the EBI has long been known as the niche for erythroid precursors, significant advancements in biology research technologies, including optimization of EBI enrichment protocols, single-cell ribonucleic acid sequencing, and imaging flow cytometry, have recently revealed that granulocytic precursors co-exist in this niche, termed erythromyeloblastic island (EMBI). More importantly, the balance noted at baseline between terminal granulopoiesis and erythropoiesis within EBIs/EMBIs is altered with diseases affecting hematopoiesis, such as stress erythropoiesis and inflammatory conditions causing anemia of inflammation. The role of the EMBI niche has yet to be fully investigated mechanistically, however, a notable degree of transcriptional and cell surface marker heterogeneity has been identified for the EMBI Mϕ, implicating its plasticity and diverse function.Summary: Terminal erythropoiesis and granulopoiesis are regulated within the EMBI. Investigations of their balance within this niche in health and disease may reveal new targets for treatment of diseases of terminal hematopoiesis.","PeriodicalId":55196,"journal":{"name":"Current Opinion in Hematology","volume":"30 4","pages":"99-105"},"PeriodicalIF":3.1,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10236084/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10266573","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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

Interferon regulatory factor-8-dependent innate immune alarm senses GATA2 deficiency to alter hematopoietic differentiation and function. 干扰素调节因子-8 依赖性先天免疫警报感知 GATA2 缺乏，从而改变造血分化和功能。

IF 3.1 3区医学

Current Opinion in Hematology Pub Date : 2023-07-01 Epub Date: 2023-04-27 DOI: 10.1097/MOH.0000000000000763

Kirby D Johnson, Mabel M Jung, Vu L Tran, Emery H Bresnick

{"title":"Interferon regulatory factor-8-dependent innate immune alarm senses GATA2 deficiency to alter hematopoietic differentiation and function.","authors":"Kirby D Johnson, Mabel M Jung, Vu L Tran, Emery H Bresnick","doi":"10.1097/MOH.0000000000000763","DOIUrl":"10.1097/MOH.0000000000000763","url":null,"abstract":"Purpose of review: Recent discoveries have provided evidence for mechanistic links between the master regulator of hematopoiesis GATA2 and the key component of interferon and innate immunity signaling pathways, interferon-regulatory factor-8 (IRF8). These links have important implications for the control of myeloid differentiation in physiological and pathological states.Recent findings: GATA2 deficiency resulting from loss of the Gata2 -77 enhancer in progenitors triggers an alarm that instigates the transcriptional induction of innate immune signaling and distorts a myeloid differentiation program. This pathological alteration renders progenitors hyperresponsive to interferon γ, toll-like receptor and interleukin-6 signaling and impaired in granulocyte-macrophage colony-stimulating factor signaling. IRF8 upregulation in -77-/- progenitors promotes monocyte and dendritic cell differentiation while suppressing granulocytic differentiation. As PU.1 promotes transcription of Irf8 and other myeloid and B-lineage genes, GATA2-mediated repression of these genes opposes the PU.1-dependent activating mechanism.Summary: As GATA2 deficiency syndrome is an immunodeficiency disorder often involving myelodysplastic syndromes and acute myeloid leukemia, elucidating how GATA2 commissions and decommissions genome activity and developmental regulatory programs will unveil mechanisms that go awry when GATA2 levels and/or activities are disrupted.","PeriodicalId":55196,"journal":{"name":"Current Opinion in Hematology","volume":"30 4","pages":"117-123"},"PeriodicalIF":3.1,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10236032/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10266574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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

Erythrocyte pyruvate kinase activation in red cell disorders. 红细胞丙酮酸激酶在红细胞紊乱中的激活。

IF 3.2 3区医学

Current Opinion in Hematology Pub Date : 2023-05-01 DOI: 10.1097/MOH.0000000000000758

Alessandro Matte, Enrica Federti, Lucia De Franceschi

{"title":"Erythrocyte pyruvate kinase activation in red cell disorders.","authors":"Alessandro Matte, Enrica Federti, Lucia De Franceschi","doi":"10.1097/MOH.0000000000000758","DOIUrl":"https://doi.org/10.1097/MOH.0000000000000758","url":null,"abstract":"Purpose of review: In red cells, pyruvate kinase is a key enzyme in the final step of glycolytic degradative process, which generates a constant energy supply via ATP production. This commentary discusses recent findings on pyruvate kinase activators as new therapeutic option in hereditary red cell disorders such as thalassemic syndromes or sickle cell disease (SCD).Recent findings: Mitapivat and etavopivat are two oral pyruvate kinase activators. Studies in a mouse model for β thalassemia have shown beneficial effects of mitapivat on both red cell survival and ineffective erythropoiesis, with an amelioration of iron homeostasis. This was confirmed in a proof-of-concept study in patients with nontransfusion-dependent thalassemias. Both mitapivat and etavopivat have been evaluated in mouse models for SCD, showing an increased 2-3DPG/ATP ratio and a reduction in haemolysis as well as in sickling. These data were confirmed in proof-of-concept clinical studies with both molecules carried in patients with SCD.Summary: Preclinical and clinical evidence indicate that pyruvate kinase activators represent new therapeutic option in hemoglobinopathies or SCD. Other red cell disorders such as hereditary spherocytosis or hereditary anaemias characterized by defective erythropoiesis might represent additional areas to investigate the therapeutic impact of pyruvate kinase activators.","PeriodicalId":55196,"journal":{"name":"Current Opinion in Hematology","volume":"30 3","pages":"93-98"},"PeriodicalIF":3.2,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10259003","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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: 2

Editorial introduction. 编辑介绍。

IF 3.2 3区医学

Current Opinion in Hematology Pub Date : 2023-05-01 DOI: 10.1097/MOH.0000000000000759

引用次数: 0

Editorial: Current Opinion in Hematology: new editor announcement. 社论：血液学当前观点：新编辑公告。

IF 3.2 3区医学

Current Opinion in Hematology Pub Date : 2023-05-01 DOI: 10.1097/MOH.0000000000000761

Sarah J Booth

引用次数: 0

Navigating the marrow sea towards erythromyeloblastic islands under normal and inflammatory conditions. 在正常和炎症条件下，在髓海中寻找红细胞母细胞岛。

IF 3.2 3区医学

Current Opinion in Hematology Pub Date : 2023-05-01 Epub Date: 2023-01-17 DOI: 10.1097/MOH.0000000000000756

Rachel Josselsohn, Betsy J Barnes, Theodosia A Kalfa, Lionel Blanc

{"title":"Navigating the marrow sea towards erythromyeloblastic islands under normal and inflammatory conditions.","authors":"Rachel Josselsohn, Betsy J Barnes, Theodosia A Kalfa, Lionel Blanc","doi":"10.1097/MOH.0000000000000756","DOIUrl":"10.1097/MOH.0000000000000756","url":null,"abstract":"Purpose of review: Terminal erythroid differentiation occurs in specialized niches called erythroblastic islands. Since their discovery in 1958, these niches have been described as a central macrophage surrounded by differentiating erythroblasts. Here, we review the recent advances made in the characterization of these islands and the role they could play in anaemia of inflammation.Recent findings: The utilization of multispectral imaging flow cytometry (flow cytometry with microscopy) has enabled for a more precise characterization of the niche that revealed the presence of maturing granulocytes in close contact with the central macrophage. These erythromyeloblastic islands (EMBIs) can adapt depending on the peripheral needs. Indeed, during inflammation wherein inflammatory cytokines limit erythropoiesis and promote granulopoiesis, EMBIs present altered structures with increased maturing granulocytes and decreased erythroid precursors.Summary: Regulation of the structure and function of the EMBI in the bone marrow emerges as a potential player in the pathophysiology of acute and chronic inflammation and its associated anaemia.","PeriodicalId":55196,"journal":{"name":"Current Opinion in Hematology","volume":"30 3","pages":"80-85"},"PeriodicalIF":3.2,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10065913/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10633092","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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

Hidden behind thromboinflammation: revealing the roles of von Willebrand factor in sickle cell disease pathophysiology. 隐藏在血栓炎症背后:揭示血管性血友病因子在镰状细胞病病理生理中的作用。

IF 3.2 3区医学

Current Opinion in Hematology Pub Date : 2023-05-01 Epub Date: 2023-01-12 DOI: 10.1097/MOH.0000000000000755

Eudorah F Vital, Wilbur A Lam

{"title":"Hidden behind thromboinflammation: revealing the roles of von Willebrand factor in sickle cell disease pathophysiology.","authors":"Eudorah F Vital, Wilbur A Lam","doi":"10.1097/MOH.0000000000000755","DOIUrl":"10.1097/MOH.0000000000000755","url":null,"abstract":"Purpose of review: This review provides an update on the pathophysiology of sickle cell disease (SCD) with a particular focus on the dysregulation of the von Willebrand factor (VWF) - ADAMTS13 axis that contributes to its pathogenesis. In discussing recent developments, we hope to encourage new and ongoing discussions surrounding therapeutic targets for SCD.Recent findings: Within the last 5 years, the role of VWF in the pathophysiology of SCD has been further elucidated and is now a target of study in ongoing clinical trials.Summary: The pathophysiology of SCD is multifaceted, as it involves systemwide vascular activation, altered blood rheology, and the activation of immune responses and coagulative pathways. The presence of VWF in excess in SCD, particularly in its largest multimeric form, greatly contributes to its pathogenesis. Understanding the molecular mechanisms that underly the presence of large VWF multimers in SCD will provide further insight into the pathogenesis of SCD and provide specific targets for therapy.","PeriodicalId":55196,"journal":{"name":"Current Opinion in Hematology","volume":"30 3","pages":"86-92"},"PeriodicalIF":3.2,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d8/37/cohem-30-86.PMC10065920.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10267001","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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: 1

VEXAS: where do we stand 2 years later? 维克斯:两年后我们的情况如何?

IF 3.2 3区医学

Current Opinion in Hematology Pub Date : 2023-03-01 DOI: 10.1097/MOH.0000000000000750

Pierre Sujobert, Maël Heiblig, Yvan Jamilloux

{"title":"VEXAS: where do we stand 2 years later?","authors":"Pierre Sujobert, Maël Heiblig, Yvan Jamilloux","doi":"10.1097/MOH.0000000000000750","DOIUrl":"https://doi.org/10.1097/MOH.0000000000000750","url":null,"abstract":"Purpose of review: Two years after the recognition of VEXAS (for Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, we propose an extensive review of the current understanding of VEXAS pathophysiology and therapeutic options.Recent findings: Among the nearly 150 articles published about VEXAS, some have provided determinant insights into VEXAS pathophysiology and treatment. Clinical data from retrospective series support the JAK inhibitor ruxolitinib as the most efficient strategy to control inflammation, and interesting results were also described with azacytidine. Allogeneic stem cell transplantation remains the only curative option, but should be proposed to carefully selected patients.Summary: Although waiting for more robust evidence from prospective clinical trials, therapeutic options emerge from retrospective studies. We propose a set of criteria that should be systematically reported to harmonize the evaluation of therapeutic outcomes. This will allow the collection of high-quality data and facilitate their subsequent meta-analysis with the overall aim of improving the management of VEXAS patients.","PeriodicalId":55196,"journal":{"name":"Current Opinion in Hematology","volume":"30 2","pages":"64-69"},"PeriodicalIF":3.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10633099","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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: 3

Is it the time to integrate novel sequencing technologies into clinical practice? 现在是将新型测序技术融入临床实践的时候了吗？

IF 3.1 3区医学

Current Opinion in Hematology Pub Date : 2023-03-01 Epub Date: 2022-12-30 DOI: 10.1097/MOH.0000000000000754

Jennifer VanOudenhove, Stephanie Halene, Lourdes Mendez

{"title":"Is it the time to integrate novel sequencing technologies into clinical practice?","authors":"Jennifer VanOudenhove, Stephanie Halene, Lourdes Mendez","doi":"10.1097/MOH.0000000000000754","DOIUrl":"10.1097/MOH.0000000000000754","url":null,"abstract":"Purpose of review: The aim of this study was to provide insight into how novel next-generation sequencing (NGS) techniques are set to revolutionize clinical practice.Recent findings: Advances in sequencing technologies have focused on improved capture of mutations and reads and cellular resolution. Both short and long read DNA sequencing technology are being refined and combined in novel ways with other multiomic approaches to gain unprecedented biological insight into disease. Single-cell (sc)DNA-seq and integrated scDNA-seq with immunophenotyping provide granular information on disease composition such as clonal hierarchy, co-mutation status, zygosity, clonal diversity and genotype phenotype correlations. These and other techniques can identify rare cell populations providing the opportunity for increased sensitivity in measurable residual disease monitoring and precise characterization of residual clones permitting distinction of leukemic from pre/nonmalignant clones.Summary: Increasing genetics-based mechanistic insights and classification of myeloid diseases along with a decrease in the cost of high-throughput NGS mean novel sequencing technologies are closer to being a reality in standard clinical practice. These technologies are poised to improve diagnostics, our ability to monitor treatment response and minimal residual disease and allow the study of premalignant conditions such as clonal haematopoiesis.","PeriodicalId":55196,"journal":{"name":"Current Opinion in Hematology","volume":"30 2","pages":"70-77"},"PeriodicalIF":3.1,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10266076","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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 fifth edition of the World Health Organization Classification and the International Consensus Classification of myeloid neoplasms: evolving guidelines in the molecular era with practical implications. 世界卫生组织分类和髓系肿瘤国际共识分类第五版:分子时代具有实际意义的发展指南。

IF 3.2 3区医学

Current Opinion in Hematology Pub Date : 2023-03-01 DOI: 10.1097/MOH.0000000000000748

Gang Zheng, Peng Li, Xiaohui Zhang, Zenggang Pan

{"title":"The fifth edition of the World Health Organization Classification and the International Consensus Classification of myeloid neoplasms: evolving guidelines in the molecular era with practical implications.","authors":"Gang Zheng, Peng Li, Xiaohui Zhang, Zenggang Pan","doi":"10.1097/MOH.0000000000000748","DOIUrl":"https://doi.org/10.1097/MOH.0000000000000748","url":null,"abstract":"Purpose of review: There have been major advances in our understanding of molecular pathogenesis of myeloid neoplasms, which prompt the updates in the classification of myeloid neoplasms in the fifth edition of World Health Organization Classification (WHO-5) and the new International Consensus Classification (ICC). The purpose of this review is to provide an overview of these two classification systems for myeloid neoplasms.Recent findings: The definition, classification, and diagnostic criteria in many myeloid entities have been refined in WHO-5 and ICC with improved understanding of morphology and integration of new genetic findings. Particularly, molecular and cytogenetic studies have been increasingly incorporated into the classification, risk stratification, and selection of therapy of myeloid neoplasms. Overall, despite some revisions and discrepancies between WHO-5 and ICC, the major categories of myeloid neoplasms remain the same. Further validation studies are warranted to fine-tune and, ideally, integrate these two classifications.Summary: Integration of clinical information, laboratory parameters, morphologic features, and cytogenetic and molecular studies is essential for the classification of myeloid neoplasms, as recommended by both WHO-5 and ICC.","PeriodicalId":55196,"journal":{"name":"Current Opinion in Hematology","volume":"30 2","pages":"53-63"},"PeriodicalIF":3.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10616030","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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: 2