Thrombosis research最新文献

{"title":"Is primary hemostasis involved in bleeding diathesis in patients with type 1 glycogen storage disease?","authors":"Floriane Devaux ,&nbsp;Claire Auditeau ,&nbsp;Cécile Bally ,&nbsp;Tiffany Pascreau ,&nbsp;Anais Brassier ,&nbsp;Myriam Dao ,&nbsp;Jean Baptiste Arnoux ,&nbsp;Oliver Wong ,&nbsp;Dominique Lasne ,&nbsp;Pascale de Lonlay ,&nbsp;Annie Harroche ,&nbsp;Delphine Borgel","doi":"10.1016/j.thromres.2025.109424","DOIUrl":"10.1016/j.thromres.2025.109424","url":null,"abstract":"<div><h3>Objectives</h3><div>Glycogen storage disease type I (GSD-I) is a metabolic disease associated with a bleeding tendency. Although decreased von Willebrand factor (VWF) and/or impaired platelet function have been reported in this condition, no direct link with bleeding has been demonstrated. The aim of this retrospective study was to assess the correlation between primary hemostasis abnormalities and haemorrhagic diathesis in a cohort of 19 GSD-I patients.</div></div><div><h3>Methods</h3><div>A Standardized International Society of Thrombosis and Hemostasis Bleeding Assessment Tool (ISTH-BAT) score was calculated for all patients to assess their bleeding history. Primary hemostasis was investigated by platelet closure time, VWF activity and antigen levels, and platelet aggregation.</div></div><div><h3>Results</h3><div>Seven of the 19 patients (37 %) had a positive ISTH-BAT score for age. Bleeding symptoms were essentially mucocutaneous. Thirteen patients (76 %) had a prolonged closure time with no significant difference whether ISTH-BAT was positive (ISTH-BATpos group) or negative (ISTH-BATneg group) (<em>p</em> = 0.60). Two patients in the ISTH-BATpos group had a moderate decrease in VWF levels. However, no statistical difference was observed between ISTH-BATpos and ISTH-BATneg groups regarding VWF activity (<em>p</em> = 0.40) or antigen (<em>p</em> = 0.82) levels either. Four patients had defective platelet aggregation, mainly in response to ADP agonist, with no significant difference between the two groups (<em>p</em> = 0.12).</div></div><div><h3>Conclusion</h3><div>As expected, GSD-I was found to be associated with an increased risk of mucocutaneous haemorrhage. However, no correlation was observed between primary hemostasis impairment and bleeding tendency in the present study.</div></div>","PeriodicalId":23064,"journal":{"name":"Thrombosis research","volume":"253 ","pages":"Article 109424"},"PeriodicalIF":3.4,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144842868","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}

{"title":"Erythrocyte rheology is altered in haemophilia patients with advanced arthropathy. A case-control study","authors":"Fabian Tomschi ,&nbsp;Marijke Grau ,&nbsp;Wilhelm Bloch ,&nbsp;Thomas Hilberg","doi":"10.1016/j.thromres.2025.109423","DOIUrl":"10.1016/j.thromres.2025.109423","url":null,"abstract":"<div><h3>Background</h3><div>Haemophilia goes along with a lack of clotting factors, which can lead to bleedings mostly being of intraarticular nature. These patients (PwH) suffer from health implications attributed to the resulting haemophilc arthropathy. Even though the haemostatic system is widely studied in PwH, little is known about red blood cell (RBC) rheology. This study aims to explore RBC rheology focussing on deformability and aggregation in PwH and to describe potential influencing factors.</div></div><div><h3>Methods</h3><div>27 PwH and 27 healthy controls (HC) were included in this study. RBC deformability (EI_max, SS½, SS½/EI_max ratio) and aggregation (AI, Shear stress at disc min) were measured using ektacytometry and obtained from syllectograms. Haematological parameters were determined. The Haemophilia Joint Health Score (HJHS) was assessed indicating musculoskeletal impairment. Pain and pain sensitivity were measured using numeric rating scales and pressure algometry. Subjective physical functioning was assessed via questionnaires.</div></div><div><h3>Results</h3><div>PwH possess higher HJHS scores than HC (<em>p</em> &lt; .001) with a range of 11–69 points. All RBC deformability parameters (<em>p</em> ≤ .007) and Shear stress at disc min (<em>p</em> &lt; .001) are impaired in PwH compared to HC. Regarding haematological parameters, only red cell distribution width (<em>p</em> = .049) and haemoglobin (<em>p</em> = .024) were different. PwH reveal higher pain states (<em>p</em> ≤ .002) and lower physical functioning (<em>p</em> &lt; .001).</div></div><div><h3>Conclusions</h3><div>This study is the first to investigate RBC rheology in PwH with haemophilic arthropathy and shows that RBC rheological properties, particularly deformability, are impaired in this population. These observations might be attributed to advanced joint restrictions and higher pain states in PwH resulting in lower physical activity as well as to present co-morbidities and co-medication.</div></div>","PeriodicalId":23064,"journal":{"name":"Thrombosis research","volume":"253 ","pages":"Article 109423"},"PeriodicalIF":3.4,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144779369","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}

{"title":"Anticoagulation therapy for patients with atrial fibrillation at primary care facilities in Beijing, China, 2017–2022","authors":"Mengyuan Fu ,&nbsp;Kexin Ling ,&nbsp;Zhiwen Gong ,&nbsp;Shujie Dong ,&nbsp;Huangqianyu Li ,&nbsp;Fang Wang ,&nbsp;Yang Xu ,&nbsp;Luwen Shi ,&nbsp;Joseph S. Ross ,&nbsp;Xiaodong Guan","doi":"10.1016/j.thromres.2025.109417","DOIUrl":"10.1016/j.thromres.2025.109417","url":null,"abstract":"","PeriodicalId":23064,"journal":{"name":"Thrombosis research","volume":"253 ","pages":"Article 109417"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757088","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}

{"title":"Corrigendum to “Quantitative protein mass spectrometry for multiplex measurement of coagulation and fibrinolytic proteins towards clinical application: What, why and how?”, [Thromb. Res. 241 (2024) 109090]","authors":"Eleonora Camilleri ,&nbsp;Mirjam Kruijt ,&nbsp;Paul L. den Exter ,&nbsp;Suzanne C. Cannegieter ,&nbsp;Nienke van Rein ,&nbsp;Yassene Mohammed ,&nbsp;Frits R. Rosendaal ,&nbsp;Christa M. Cobbaert ,&nbsp;Bart J.M. van Vlijmen ,&nbsp;L. Renee Ruhaak","doi":"10.1016/j.thromres.2025.109416","DOIUrl":"10.1016/j.thromres.2025.109416","url":null,"abstract":"","PeriodicalId":23064,"journal":{"name":"Thrombosis research","volume":"253 ","pages":"Article 109416"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144749392","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}

{"title":"Efficacy and safety of direct oral anticoagulants compared to no anticoagulation for stroke prevention in atrial fibrillation patients with prior intracranial hemorrhage: A systematic review and meta-analysis","authors":"Muhammad Ibrahim ,&nbsp;Hassan Abdullah ,&nbsp;Aroosa Zafar ,&nbsp;Akash Kumar ,&nbsp;Fatima Ajmal ,&nbsp;Daniyal Talib ,&nbsp;Hamza Shabbir ,&nbsp;Mahnoor Shah ,&nbsp;Malaika Sardar ,&nbsp;Sarmad Masaud ,&nbsp;Mehrosh Zafar ,&nbsp;Zoya Habib ,&nbsp;F.N.U. Sagar ,&nbsp;Fatima Zahra","doi":"10.1016/j.thromres.2025.109418","DOIUrl":"10.1016/j.thromres.2025.109418","url":null,"abstract":"","PeriodicalId":23064,"journal":{"name":"Thrombosis research","volume":"253 ","pages":"Article 109418"},"PeriodicalIF":3.4,"publicationDate":"2025-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757313","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}

{"title":"Antithrombotic therapy for lower limb peripheral arterial disease: A deep dive into guideline quality and recommendation diversity","authors":"Hong-Yan Li ,&nbsp;Tao Wang ,&nbsp;Jing Wang ,&nbsp;Feng Cheng ,&nbsp;Li-Hong Wang ,&nbsp;Hai-Shan Wang","doi":"10.1016/j.thromres.2025.109414","DOIUrl":"10.1016/j.thromres.2025.109414","url":null,"abstract":"<div><h3>Background</h3><div>This study systematically evaluates clinical practice guidelines (CPGs) addressing antithrombotic management in lower limb peripheral arterial disease (PAD), aiming to synthesize evidence-based insights for clinical decision-making.</div></div><div><h3>Methods</h3><div>We systematically identified CPGs published between January 2020 and January 2025 from electronic databases, guideline repositories, and professional association websites. Guideline quality and recommendation validity were assessed using the Appraisal of Guidelines for Research &amp; Evaluation II (AGREE II) and the Appraisal of Guidelines for Research and Evaluation-Recommendation Excellence (AGREE-REX), respectively. Antithrombotic recommendations were extracted and analyzed for consensus and divergence.</div></div><div><h3>Results</h3><div>11 CPGs met the inclusion criteria. The AGREE II evaluations identified seven high-quality CPGs. The median (interquartile range [IQR]) scores for the AGREE II six domains were as follows: scope and purpose, 88.9 % (16.7 %); stakeholder involvement, 84.7 % (34.7 %); rigour of development, 85.4 % (47.9 %); clarity of presentation, 90.3 % (8.3 %); applicability, 77.1 % (40.6 %); and editorial independence, 85.4 % (33.3 %). For AGREE-REX, the domain scores were clinical applicability, 75.0 % (17.0 %); values and preferences, 29.2 % (15.0 %); and implementability, 41.7 % (15.0 %). A total of 148 key recommendations were extracted. Antiplatelet therapy remains contentious for asymptomatic PAD management. Current guidelines increasingly recommend low-dose rivaroxaban (2.5 mg twice daily) plus aspirin for symptomatic PAD, supported by recent cardiovascular outcome trials.</div></div><div><h3>Conclusion</h3><div>Current PAD guidelines demonstrate variable methodological quality, necessitating improved rigour of development processes and implementation strategies. Future CPGs should strictly adhere to standardized methodologies and prioritize effective dissemination. Different guidelines present varying opinions on antithrombotic treatment strategies. Recommendations should be periodically revised as new evidence becomes available.</div></div>","PeriodicalId":23064,"journal":{"name":"Thrombosis research","volume":"253 ","pages":"Article 109414"},"PeriodicalIF":3.4,"publicationDate":"2025-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144749391","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}

{"title":"Investigating the impact of platelet transfusions on adverse outcomes in patients with thrombotic thrombocytopenic purpura: A systematic review of published studies","authors":"Bianca Clerici ,&nbsp;Elisa Tarasconi ,&nbsp;Chiara Benedetto ,&nbsp;Chiara Pisetta ,&nbsp;Simone Birocchi ,&nbsp;Gian Marco Podda","doi":"10.1016/j.thromres.2025.109413","DOIUrl":"10.1016/j.thromres.2025.109413","url":null,"abstract":"","PeriodicalId":23064,"journal":{"name":"Thrombosis research","volume":"253 ","pages":"Article 109413"},"PeriodicalIF":3.4,"publicationDate":"2025-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757312","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}

{"title":"Characterization of a novel factor X variant, p.F139L, associated with bleedings in heterozygous and compound heterozygous form","authors":"Marit Hellum ,&nbsp;Marit Sletten ,&nbsp;Marie S. Le ,&nbsp;Vidar Stavseth ,&nbsp;Heidi Glosli ,&nbsp;Paul Hoff Backe ,&nbsp;Nina Iversen ,&nbsp;Carola E. Henriksson","doi":"10.1016/j.thromres.2025.109412","DOIUrl":"10.1016/j.thromres.2025.109412","url":null,"abstract":"<div><h3>Introduction</h3><div>Congenital factor X (FX) deficiency is a rare bleeding disorder. Heterozygous patients may experience bleedings like epistaxis or bleeding complications after dental extraction and surgery, whereas homozygous individuals often experience more severe bleedings.</div></div><div><h3>Aim</h3><div>To characterize a new FX variant found in two unrelated probands with mild to moderate bleeding tendencies.</div></div><div><h3>Methods</h3><div>Prothrombin time (PT) Quick, APTT, FX activity, FX antigen, thrombin generation and DNA sequencing were performed for probands and their families. <em>F10</em> variants were explored in programs for <em>in silico</em> predictions and structural analyses, and <em>in vitro</em> expression was used for further characterization.</div></div><div><h3>Results</h3><div>A novel missense variant (c.415T&gt;C, p.F139L) was found in heterozygous form in proband 1 and in compound heterozygous form in proband 2, in combination with a previously known missense variant (c.424G&gt;A, p.E142K). All individuals carrying the p.F139L variant had borderline/prolonged PT Quick (11.9–15.9 s) and APTT (36–43 s) and reduced thrombin generation. Both FX antigen (30–51 %) and activity (37–63 IU/dL) were reduced, indicating a type I deficiency. Structural analysis indicated that interactions between the EGF-2 and the protease domain of FX could be disrupted in the p.F139L variant, causing destabilization of the protein. The p.E142K variant was predicted to have less impact on FX structure. <em>In vitro</em> expression results further supported plasma findings.</div></div><div><h3>Conclusion</h3><div>We report a new FX variant, p.F139L, causing a type I FX deficiency associated with mild to moderate bleedings in heterozygous and compound heterozygous form. The variant causes structural changes which likely affect intracellular processing of FX.</div></div>","PeriodicalId":23064,"journal":{"name":"Thrombosis research","volume":"253 ","pages":"Article 109412"},"PeriodicalIF":3.4,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144766528","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}

{"title":"Anticoagulant therapy for cancer-associated splanchnic vein thrombosis: Outcomes during a one-year follow-up period","authors":"Adrian Garcia-Villa ,&nbsp;Juan José Criado-Álvarez ,&nbsp;María Carnevali ,&nbsp;Mario Aramberri ,&nbsp;Carme Font ,&nbsp;Carmen Díaz-Pedroche","doi":"10.1016/j.thromres.2025.109411","DOIUrl":"10.1016/j.thromres.2025.109411","url":null,"abstract":"<div><h3>Introduction</h3><div>The incidence of splanchnic vein thrombosis (SVT) in cancer patients has increased in recent years due to improved survival rates and the widespread use of scheduled radiological tests. However, studies describing the long-term prognosis and benefits of anticoagulant treatment are scarce. The objectives were to describe long-term prognosis and to compare outcomes based on anticoagulant treatment during a one-year follow-up.</div></div><div><h3>Material and methods</h3><div>This was a retrospective observational study of consecutive patients hospitalized between 2015 and 2020 with a diagnosis of cancer-associated SVT either prior to admission or during hospitalization.</div></div><div><h3>Results</h3><div>The study included 201 patients, with intra-abdominal tumors (78.2 %) predominating, particularly pancreatic cancer (43.8 %). Portal thrombosis (58.6 %) was the most common type of SVT. Approximately 41.3 % of cases received anticoagulant treatment at the diagnosis of SVT. The overall survival rate at 12 months was 39.7 %. Anticoagulant therapy was associated with a significantly higher accumulated incidence of recanalization and bleeding. However, no differences were found in the survival and recurrence of thrombosis. Tumor thrombus (OR 2.44; CI 95 % 1.32–4.52) and the metastatic status of the oncological disease (OR 3.07; CI 95 % 1.63–5.8) were predictive factors for death.</div></div><div><h3>Conclusions</h3><div>SVT is a common complication in cancer patients, particularly in those with abdominal tumors. Tumor thrombus and metastatic status were negative prognostic factors. Anticoagulant treatment was associated with a higher rate of recanalization as well as a higher risk of bleeding, without benefits on survival or prevention of thrombosis recurrence.</div></div>","PeriodicalId":23064,"journal":{"name":"Thrombosis research","volume":"253 ","pages":"Article 109411"},"PeriodicalIF":3.4,"publicationDate":"2025-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144721008","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}

{"title":"JAK2V617F-positive extracellular genomic materials drive endothelial inflammation and thrombosis in myeloproliferative neoplasms","authors":"Selin Fulya Toprak ,&nbsp;Cemal Çağıl Koçana ,&nbsp;Esra Nur Demirtaş ,&nbsp;Gülşah Tuna ,&nbsp;İldeniz Uslu-Bıçak ,&nbsp;Büşra Yaşa-Çevik ,&nbsp;Selçuk Sözer","doi":"10.1016/j.thromres.2025.109407","DOIUrl":"10.1016/j.thromres.2025.109407","url":null,"abstract":"<div><div>Endothelial cells (ECs) play a pivotal role in vascular inflammation and thrombosis, central processes in myeloproliferative neoplasms (MPNs). Vascular complications, particularly thrombosis, contribute significantly to MPN morbidity and mortality; however, the molecular mechanisms underlying endothelial dysfunction remain incompletely understood.</div><div>In this study, we investigated the effects of <em>JAK2</em>V617F-positive extracellular genomic materials (EGMs)—including cell-free DNA (cfDNA), and exosomes, derived from HEL cells—on endothelial inflammation, coagulation, and nucleic acid-sensing pathways to elucidate their contribution to MPN-associated vascular pathology. Human umbilical vein endothelial cells (HUVECs) were treated with <em>JAK2</em>V617F-positive EGMs and culture supernatant (CS). We assessed proliferation, apoptosis, <em>HMGB1</em> expression and release, cytokine secretion, nucleic acid-sensing pathway activation, and coagulation-related gene expression via RT-PCR, ELISA, and flow cytometry.</div><div>We have demonstrated that exosomes exhibited the most potent effects on endothelial activation, robust HMGB1 secretion and cytokine release, including IL-1β, IL-6, and MCP-1, indicating activation of early inflammation while cfDNA induced intracellular HMGB1 accumulation with limited extracellular release. We further demonstrate that <em>JAK2</em>V617F-positive EGMs activate nucleic acid sensing pathways (cGAS-STING and TLR9), leading to increased expression of proinflammatory mediators such as <em>NF-κB</em>, <em>IRF3</em>, and <em>IRF7</em>. The upregulation of coagulation-related genes (<em>F10, F3</em>, <em>F2RL1</em>) and adhesion molecules (<em>ICAM-1, TF</em>) confirmed that EGM exposure promotes a pro-thrombotic phenotype in ECs, mirroring the hypercoagulable state in MPN patients.</div><div>As our in vitro system lacks physiological conditions such as blood flow dynamics and immune cell interactions, further validation in patient-derived samples and in vivo models with varying <em>JAK2</em>V617F allele burdens is required. Nonetheless, these findings establish a mechanistic link between oncogenic EGMs and vascular complications in MPNs, highlighting their role in chronic inflammation and thrombosis. Targeting EGM-mediated endothelial dysfunction may offer a novel therapeutic approach to mitigate vascular complications in MPN patients.</div></div>","PeriodicalId":23064,"journal":{"name":"Thrombosis research","volume":"253 ","pages":"Article 109407"},"PeriodicalIF":3.4,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144724481","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}