Blood Vessels, Thrombosis & Hemostasis最新文献

{"title":"Hematologists’ comfort and experiences with providing care to transgender youth","authors":"Eric S. Mullins ,&nbsp;Tanya L. Kowalczyk Mullins","doi":"10.1016/j.bvth.2025.100054","DOIUrl":"10.1016/j.bvth.2025.100054","url":null,"abstract":"<div><h3>Abstract</h3><div>Transgender and gender-diverse (TG) people with preexisting risk factors for thrombosis may seek hematologic evaluation before starting gender-affirming hormone therapy (GAHT). Because no formal guidelines on management of thrombosis risk exist, variations in management are likely to occur. We characterized hematologists’ experience and comfort with caring for TG youth and explored experiences with recommending thromboprophylaxis before GAHT. Hematologists caring for youth aged ≤22 years and practicing in the midwestern United States completed semistructured interviews assessing demographics, practice characteristics, comfort with caring for TG people, education in TG clinical care, suggested interventions to improve comfort, and experiences with recommending and/or prescribing thromboprophylaxis before GAHT. Of the 15 hematologists interviewed (12 pediatric, 2 adult, and 1 dual trained), nearly all had cared for TG adolescents (n = 12) or young adults (n = 14). Participants reported variable comfort with asking about name and pronouns and knowledge about the gender transition process. Although most hematologists reported having had some education about TG clinical care, this primarily occurred after formal training was completed. Suggested interventions to increase comfort with caring for TG youth included educating hematologists about gender care, changes in the electronic medical record, and more data on thrombosis risk associated with GAHT. One-third of participants had recommended and started thromboprophylaxis for patients before GAHT. Five additional hematologists had evaluated youths before GAHT but had not recommended thromboprophylaxis. Because hematologists are evaluating patients for potential thromboprophylaxis before GAHT, education about caring for TG people and data about thrombosis risk are needed to improve care for this population.</div></div>","PeriodicalId":100190,"journal":{"name":"Blood Vessels, Thrombosis & Hemostasis","volume":"2 2","pages":"Article 100054"},"PeriodicalIF":0.0,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143601656","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiovascular risk factors in myeloproliferative neoplasms: associations with survival and thrombotic outcomes","authors":"Joan How ,&nbsp;Orly Leiva ,&nbsp;Robert Redd ,&nbsp;Anna E. Marneth ,&nbsp;Daniel J. DeAngelo ,&nbsp;Christina M. Dieli-Conwright ,&nbsp;Areej El-Jawahri ,&nbsp;Baransal Kamaz ,&nbsp;Chulwoo Kim ,&nbsp;R. Coleman Lindsley ,&nbsp;Marlise Luskin ,&nbsp;Maximilian Stahl ,&nbsp;Mohammed Wazir ,&nbsp;Lachelle D. Weeks ,&nbsp;Gabriela S. Hobbs","doi":"10.1016/j.bvth.2025.100051","DOIUrl":"10.1016/j.bvth.2025.100051","url":null,"abstract":"<div><h3>Abstract</h3><div>Cardiovascular risk factors (CVRFs) are important modifiers of thrombosis in patients with essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF). We performed a retrospective cohort analysis evaluating CVRFs in 1005 patients with myeloproliferative neoplasms (MPNs) from the Dana-Farber Cancer Institute Hematologic Malignancies Data Repository from 2014 to 2023. We also included a non-MPN group of 1543 age- and sex-matched controls with no known diagnoses of hematologic malignancies to evaluate whether CVRFs differentially affected outcomes. CVRFs were identified through International Classification of Diseases codes for hypertension, hyperlipidemia, type 2 diabetes mellitus (DM2), current smoking, or body mass index ≥30 before MPN diagnosis. CVRFs occurred in 34% of patients with MPNs. Patients with MPN with ≥1 CVRF had increased risk of death (hazard ratio [HR], 2.52; 95% confidence interval [CI], 1.9-3.35) and arterial/venous thrombosis (HR, 3.05; 95% CI 2.39-3.92). Within MPN subtypes, patients with ET, PV, and MF who had CVRFs also demonstrated worse overall survival and thrombotic outcomes. Among CVRFs, only DM2 predicted worse thrombotic outcomes in patients with MPNs. The HR of CVRF on thrombosis was decreased in patients with MPNs compared with non-MPN controls (HR, 0.51; 95% CI, 0.36-0.86). Looking at ET, PV, and MF specifically, the presence of a CVRF also had less of an impact on thrombotic risk in ET compared with controls (HR, 0.35; <em>P</em> = .019); no interactions between MPN diagnosis and CVRFs were seen in patients with PV and MF. Our results underscore both the necessity of managing CVRFs in MPNs to improve patient morbidity and mortality and the need to ameliorate thrombotic risk with measures beyond addressing CVRFs.</div></div>","PeriodicalId":100190,"journal":{"name":"Blood Vessels, Thrombosis & Hemostasis","volume":"2 3","pages":"Article 100051"},"PeriodicalIF":0.0,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144240888","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effects of sickle trait on maternal and perinatal outcomes among pregnant women","authors":"Sarah Sewaralthahab ,&nbsp;Jiling Chou ,&nbsp;Stephen Fernandez ,&nbsp;Nawar Shara ,&nbsp;Hedy P. Smith","doi":"10.1016/j.bvth.2025.100049","DOIUrl":"10.1016/j.bvth.2025.100049","url":null,"abstract":"<div><h3>Abstract</h3><div>The risk of multiple adverse pregnancy outcomes and perinatal outcomes among pregnant women with sickle cell trait (SCT) is not known. Our objective was to compare differences in adverse outcomes, specifically pregnancy-related hypertensive disease (PRHD), pyelonephritis/urinary tract infection (UTI), and low birth weight (LBW), between pregnant women with SCT and healthy controls. This was a retrospective cohort study of women who delivered between 2015 and 2020. We included all women with SCT, that is, hemoglobin electrophoresis AS. Women with SCT were matched in a 1:2 to women without SCT, controlling for age, gravidity, and parity. Our primary outcomes were PRHD, pyelonephritis/UTI, and LBW baby. Multivariable logistic regression modeling examined the associations between patients’ characteristics and the primary outcomes.There were 162 women with SCT, and 324 healthy control women were enrolled. Bivariate analysis revealed that women with SCT had a higher proportion of PRHD (38.9% vs 34.9%; <em>P</em> = .39), pyelonephritis/UTI (11.7% vs 7.1%; <em>P</em> = .09), but a lower proportion of LBW (10.5% vs 16.0%; <em>P</em> = .1). In multivariable analysis, after controlling for confounders, SCT was not an independent predictor of PRHD. However, SCT was an independent predictor of pyelonephritis/UTI (adjusted odds ratio [aOR], 1.98; 95% confidence interval [CI], 1.02-3.85) and of a lower risk of having a LBW baby (aOR, 0.48; 95% CI, 0.25-0.94). SCT is not associated with an increased risk of PRHD. However, SCT is associated with pregnancy outcomes, including higher risk of pyelonephritis/UTI but a lower risk of LBW babies.</div></div>","PeriodicalId":100190,"journal":{"name":"Blood Vessels, Thrombosis & Hemostasis","volume":"2 2","pages":"Article 100049"},"PeriodicalIF":0.0,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143601310","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A NOTCH4 intron 28 regulatory element controls arterial specification and lymphoid development from hPSCs","authors":"Ho Sun Jung ,&nbsp;Divine Mensah Sedzro ,&nbsp;Peng Liu ,&nbsp;Igor I. Slukvin","doi":"10.1016/j.bvth.2025.100046","DOIUrl":"10.1016/j.bvth.2025.100046","url":null,"abstract":"<div><h3>Abstract</h3><div>NOTCH signaling plays a critical role in arterial specification, and the formation of transient definitive lymphomyeloid progenitors and hematopoietic stem cells from hemogenic endothelium (HE). To investigate NOTCH signaling mechanisms critical for arterial and lymphoid specification, we performed single-cell multiomics analysis of human pluripotent stem cells (hPSCs) at different stages of hematopoietic differentiation. These studies uncovered the activation of NOTCH signaling and the arterial program, along with dynamic changes in related regulons throughout the HE specification and the endothelial-to-hematopoietic transition. We revealed that expression of NOTCH4 in the arterial endothelium is controlled by a <em>cis</em>-regulatory element within intron 28 (<em>NOTCH4in28</em>) through SOX17 binding. Deletion of <em>NOTCH4in28</em> in conventional and conditional iSOX17 hPSCs impaired the formation of the early wave DLL4⁺CXCR4^+/− HE with arterial features, as well as hematopoietic progenitors with T and natural killer lymphoid potential. Collectively, we provide compelling evidence that NOTCH4 and the <em>NOTCH4in28 cis</em>-regulatory element play an important role in arterial specification and lymphoid development in hPSC cultures.</div></div>","PeriodicalId":100190,"journal":{"name":"Blood Vessels, Thrombosis & Hemostasis","volume":"2 2","pages":"Article 100046"},"PeriodicalIF":0.0,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143839058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk factors for joint bleeding in severe hemophilia A and B: analysis of the Community Counts longitudinal surveillance cohort.","authors":"Marilyn J Manco-Johnson, Binh Le, Suchitra Acharya, Sanjay Ahuja, Meera Chitlur, Divyaswathi Citla-Sridhar, Stacey Ann Fedewa, Daniel Isaac, Roshni Kulkarni, Laura A Schieve, Anjali Sharathkumar, J Michael Soucie","doi":"10.1016/j.bvth.2025.100047","DOIUrl":"10.1016/j.bvth.2025.100047","url":null,"abstract":"<p><p>Joint bleeding is the primary determinant of end-stage arthropathy in hemophilia; joint bleeding has greatly decreased with the use of prophylaxis and introduction of highly effective therapies. This study aimed to determine current risk factors for joint bleeding in persons with severe hemophilia A or B. Demographic, treatment, and bleeding outcome data from Community Counts, a US national surveillance project, were analyzed. Data were collected at annual visits between 2013 and 2022. Eligibility included factor VIII or IX of <1%, no inhibitor, age of 2 to 44 years, and on treatment with continuous prophylaxis. Annual joint bleeding rate (AJBR) differences across demographic and clinical subgroups were compared via rate ratios and 95% confidence intervals, and with multivariate methods accounting for multiple measurements over time. The analysis included 2527 males with hemophilia, 7211 observation years, and 10 046 joint bleeds. Lower AJBR in hemophilia A was most strongly associated with use of emicizumab. For both hemophilia A and B, patient-associated factors, younger age, fewer missed doses, and full employment were all independently associated with lower AJBR, as was treatment in the Northeast of the United States. The findings of this comprehensive analysis of a large, diverse sample drawn from hemophilia treatment centers across the United States, underscore the important contributions of patient, community, and treatment factors on joint outcomes in severe hemophilia. Use of emicizumab and few missed doses independently predicted low AJBR, highlighting the interplay of access and adherence to care.</p>","PeriodicalId":100190,"journal":{"name":"Blood Vessels, Thrombosis & Hemostasis","volume":"2 3","pages":"100047"},"PeriodicalIF":0.0,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12356300/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144877752","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Seasonal variation in immune thrombotic thrombocytopenic purpura in New England","authors":"Jorge N. Ruiz Lopez ,&nbsp;Ishan Tatake ,&nbsp;Pavan K. Bendapudi","doi":"10.1016/j.bvth.2025.100050","DOIUrl":"10.1016/j.bvth.2025.100050","url":null,"abstract":"","PeriodicalId":100190,"journal":{"name":"Blood Vessels, Thrombosis & Hemostasis","volume":"2 2","pages":"Article 100050"},"PeriodicalIF":0.0,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143563421","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Regression of pathological blood vessels by inhibition of mast cell function","authors":"WonKyung J. Cho ,&nbsp;Sharad K. Mittal ,&nbsp;Aastha Singh ,&nbsp;Elsayed Elbasiony ,&nbsp;Lei Xi ,&nbsp;Olufemi S. Folorunso ,&nbsp;Vinay K. Pulimamidi ,&nbsp;Sunil K. Chauhan","doi":"10.1016/j.bvth.2025.100044","DOIUrl":"10.1016/j.bvth.2025.100044","url":null,"abstract":"","PeriodicalId":100190,"journal":{"name":"Blood Vessels, Thrombosis & Hemostasis","volume":"2 2","pages":"Article 100044"},"PeriodicalIF":0.0,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143833229","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Small-molecule inhibitor screen to identify mechanisms of sickle hemoglobin clearance by liver endothelium","authors":"Tomasz W. Kaminski ,&nbsp;Hong Zhang ,&nbsp;Omika Katoch ,&nbsp;Qizhen Shi ,&nbsp;Gregory J. Kato ,&nbsp;Prithu Sundd ,&nbsp;Tirthadipa Pradhan-Sundd","doi":"10.1016/j.bvth.2025.100045","DOIUrl":"10.1016/j.bvth.2025.100045","url":null,"abstract":"<div><h3>Abstract</h3><div>Intrahepatic accumulation of cell-free hemoglobin (Hb) is a significant pathology linked with hemolytic disorders such as sickle cell disease (SCD). In addition to hepatic Kupffer cells, liver sinusoidal endothelial cells (LSECs) were recently reported to contribute to Hb clearance in SCD mice and patients via currently unknown endocytic mechanism. Using small-molecule inhibitors of endocytic pathway components in primary human and mouse LSECs, we show that sickle-Hb (HbS) uptake by LSECs occurs predominantly through micropinocytosis or fluid-phase endocytosis. However, inhibiting clathrin-mediated endocytosis, receptor recycling, or drop in pH also significantly attenuated HbS uptake by LSECs. LSEC-driven HbS uptake was independent of haptoglobin. Finally, we found that the presence of lipid droplets promotes endothelial HbS internalization, whereas hypolipidemic condition inhibits it. In conclusion, this study identifies previously unknown alternative mechanism of LSEC-mediated HbS internalization. Our findings also inform the need to evaluate the therapeutic potential of blocking these mechanisms to ameliorate hemolysis-associated liver damage in SCD and other hemolytic disorders.</div></div>","PeriodicalId":100190,"journal":{"name":"Blood Vessels, Thrombosis & Hemostasis","volume":"2 2","pages":"Article 100045"},"PeriodicalIF":0.0,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143705849","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Is it time for nitrated fibrinogen to prove its worth as a risk marker for venous thromboembolism?","authors":"Alessia Arcaro ∗ ,&nbsp;Alessio Lepore ∗ ,&nbsp;Sergio Davinelli ,&nbsp;Giovanni Scapagnini ,&nbsp;Francesco Giallauria ,&nbsp;Paul R. J. Ames ,&nbsp;Fabrizio Gentile","doi":"10.1016/j.bvth.2024.100042","DOIUrl":"10.1016/j.bvth.2024.100042","url":null,"abstract":"","PeriodicalId":100190,"journal":{"name":"Blood Vessels, Thrombosis & Hemostasis","volume":"2 2","pages":"Article 100042"},"PeriodicalIF":0.0,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143747948","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Understanding the structure of β2-glycoprotein I: new insights and future paths for antiphospholipid syndrome","authors":"Suresh Kumar ,&nbsp;Nicola Pozzi","doi":"10.1016/j.bvth.2024.100041","DOIUrl":"10.1016/j.bvth.2024.100041","url":null,"abstract":"<div><h3>Abstract</h3><div>Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent life-threatening blood clots and pregnancy complications in individuals with antiphospholipid antibodies. Among these antibodies, those targeting the plasma glycoprotein β₂-glycoprotein I (β₂GPI) hold particular clinical significance. Despite extensive research, controversies persist regarding the structure of β₂GPI, which has substantial implications for understanding autoantibody reactivity and APS development. This article critically examines recent advancements in the structural biology of β₂GPI and its relevance to the recognition of antiphospholipid antibodies. Additionally, it introduces a new structure-based theory to explain how autoantibodies interact with β₂GPI and the functional consequence of this interaction. Finally, it identifies potential areas for future research that could enhance approaches to diagnosing and treating APS.</div></div>","PeriodicalId":100190,"journal":{"name":"Blood Vessels, Thrombosis & Hemostasis","volume":"2 2","pages":"Article 100041"},"PeriodicalIF":0.0,"publicationDate":"2024-12-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143858975","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}