Seminars in thrombosis and hemostasis最新文献_第5页

Bleeding in Patients with Renal Impairment: A Current Perspective. 肾损害患者出血——当前观点。

IF 4.2 2区医学

Seminars in thrombosis and hemostasis Pub Date : 2026-09-01 Epub Date: 2025-10-27 DOI: 10.1055/a-2731-2487

Robyn Haysom, Nadir Aziz, Dawn Swan, Jecko Thachil

引用次数: 0

Genetic Characterization of Congenital Fibrinogen Disorders: A Retrospective Analysis of 102 Unrelated Patients in China. 先天性纤维蛋白原疾病的遗传特征：中国102例无相关性患者的回顾性分析。

IF 4.2 2区医学

Seminars in thrombosis and hemostasis Pub Date : 2026-09-01 Epub Date: 2025-11-26 DOI: 10.1055/a-2731-2301

Jiaoyuan Li, Na Shen, Ming Luo, Fang Peng, Yanjun Lu, Ning Tang, Xiong Wang, Dengju Li

{"title":"Genetic Characterization of Congenital Fibrinogen Disorders: A Retrospective Analysis of 102 Unrelated Patients in China.","authors":"Jiaoyuan Li, Na Shen, Ming Luo, Fang Peng, Yanjun Lu, Ning Tang, Xiong Wang, Dengju Li","doi":"10.1055/a-2731-2301","DOIUrl":"10.1055/a-2731-2301","url":null,"abstract":"Abstract: Congenital fibrinogen disorders (CFDs) result from deficiencies in the fibrinogen-encoding genes FGA, FGB, and FGG, causing either quantitative or qualitative fibrinogen abnormalities. In this study, we conducted an extensive evaluation on clinical, laboratory, and genetic characteristics of 102 CFD patients. Fibrinogen levels were determined by Clauss method and/or prothrombin time (PT)-derived method. Routine coagulation parameters including PT, activated partial thromboplastin time (APTT), and thrombin time (TT) were also assessed. Genetic mutations were detected through either next-generation sequencing or comprehensive whole-exome sequencing. The case series comprised 38 males and 64 females, with a median diagnosis age of 33 years. In patients where laboratory results were available, the function fibrinogen levels tested by Clauss method were decreased, whereas only 51.7% exhibited reduced fibrinogen concentrations by PT-derived method. A total of 55 germline mutations were identified, including 26 novel mutations not previously documented in the literature. Forty-two percent of unrelated patients were carriers of hotspot mutations. The laboratory results and clinical symptoms were highly variable among patients, even within patients harboring the same mutation. However, TT was significantly prolonged in qualitative CFDs compared with quantitative CFDs. All the patients harboring the hotspot mutations showed qualitative deficiency of fibrinogen. We also demonstrated that qualitative CFDs were particularly prone to harboring missense variants, whereas nearly all the null mutations were classified into the quantitative group. This study presents a genetic landscape of CFD patients, and their gene-phenotype relationships. The novel identified genetic variants expand the known genetic spectrum of CFDs.","PeriodicalId":21673,"journal":{"name":"Seminars in thrombosis and hemostasis","volume":" ","pages":"732-740"},"PeriodicalIF":4.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145638265","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

From Fresh Frozen Plasma to First-in-Human: Bringing Coagulation Factor V Deficiency into Therapeutic Trials. 从新鲜冷冻血浆到首次人体试验：将凝血因子V缺乏症纳入治疗试验。

IF 4.2 2区医学

Seminars in thrombosis and hemostasis Pub Date : 2026-09-01 Epub Date: 2025-10-30 DOI: 10.1055/a-2735-1659

Jeremy W Jacobs, Sheharyar Raza, Cristina A Figueroa Villalba, Brian D Adkins, Shannon C Walker, Garrett S Booth, Deva Sharma, Allison P Wheeler

{"title":"From Fresh Frozen Plasma to First-in-Human: Bringing Coagulation Factor V Deficiency into Therapeutic Trials.","authors":"Jeremy W Jacobs, Sheharyar Raza, Cristina A Figueroa Villalba, Brian D Adkins, Shannon C Walker, Garrett S Booth, Deva Sharma, Allison P Wheeler","doi":"10.1055/a-2735-1659","DOIUrl":"10.1055/a-2735-1659","url":null,"abstract":"Abstract: Congenital factor V (FV) deficiency, affecting approximately 1 in 1 million individuals worldwide, remains among the rare bleeding disorders (RBDs) without a licensed factor-specific replacement therapy. While other RBDs have successfully transitioned from plasma-based treatment to approved factor concentrates-exemplified by factor X deficiency's progression to US Food & Drug Administration (FDA)-approved Coagadex and two FDA-approved concentrates for factor XIII deficiency-FV deficiency treatment has remained unchanged for decades, relying solely on plasma and platelet transfusions. Two promising therapeutic candidates have emerged: a human plasma-derived FV concentrate demonstrating in vitro correction of severe deficiency, and an engineered activated FV (superFVa) showing potent hemostatic activity in preclinical models. This commentary outlines a pragmatic pathway to clinical trials, leveraging proven development strategies from other RBDs, existing registry infrastructure, and regulatory incentives for rare diseases. We propose phased trials combining pharmacodynamic endpoints with clinical outcomes, enabling feasible enrollment while generating decision-grade evidence. The time has come to extend modern therapeutic development to FV deficiency.","PeriodicalId":21673,"journal":{"name":"Seminars in thrombosis and hemostasis","volume":" ","pages":"746-753"},"PeriodicalIF":4.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145408991","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Lipopolysaccharide and Coagulation Factor XII: Biophysics of Contact Activation in Infection. 脂多糖与凝血因子XII：感染中接触活化的生物物理学。

IF 4.2 2区医学

Seminars in thrombosis and hemostasis Pub Date : 2026-09-01 Epub Date: 2025-10-07 DOI: 10.1055/a-2716-6782

André L Lira, Katelyn C Drew, Cristina Puy, Joseph J Shatzel, Owen J T McCarty

{"title":"Lipopolysaccharide and Coagulation Factor XII: Biophysics of Contact Activation in Infection.","authors":"André L Lira, Katelyn C Drew, Cristina Puy, Joseph J Shatzel, Owen J T McCarty","doi":"10.1055/a-2716-6782","DOIUrl":"10.1055/a-2716-6782","url":null,"abstract":"Abstract: Lipopolysaccharide (LPS), a key component of the outer membrane of Gram-negative bacteria, is well-known for its role in triggering inflammation via innate immune receptors. However, evidence suggests that LPS can influence coagulation, in part through activation of the contact pathway. Recent studies from our group and others demonstrate that the supramolecular organization and physicochemical properties of LPS-such as aggregate size, surface charge, and chemotype-critically determine the ability of LPS to activate coagulation factor XII (FXII). While monomeric LPS can modulate FXII activity, only aggregated forms of LPS (e.g., micelles) function as a procoagulant surface, initiating contact activation. This review synthesizes current knowledge on LPS structural heterogeneity and explores how the biophysical properties of LPS govern supramolecular assembly in aqueous environments, ultimately dictating interactions with the contact activation pathway. We further discuss the possible mechanisms by which LPS-driven FXII activation contributes to thromboinflammatory disorders, including disseminated intravascular coagulation and sepsis-associated vascular leakage. Finally, we highlight novel therapeutic strategies-from FXIIa inhibitors to molecules that disrupt LPS supramolecular structures-as potential interventions to mitigate coagulation-driven pathology during bacterial infections. These insights not only reflect our growing understanding of infection-associated thrombosis but may also pave the way for targeted therapies in sepsis and other thromboinflammatory conditions.","PeriodicalId":21673,"journal":{"name":"Seminars in thrombosis and hemostasis","volume":" ","pages":"683-691"},"PeriodicalIF":4.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13616133/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145245141","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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 Case Report: A 65-Year-Old Presenting with Disseminated Intravascular Coagulation as a Rituximab Infusional Reaction. 临床病例报告：一名65岁的患者以利妥昔单抗输注反应表现为弥漫性血管内凝血。

IF 4.2 2区医学

Seminars in thrombosis and hemostasis Pub Date : 2026-09-01 Epub Date: 2025-11-10 DOI: 10.1055/a-2731-6736

Elena Martínez-De Antonio, Gerard Ronda-Roca, Belén Navarro-Matilla, Almudena de Laiglesia-Lorenzo, Concepción Payares-Herrera

引用次数: 0

Vonicog Alfa versus Plasma-Derived von Willebrand Factor during Hospitalization: Results of an Observational Retrospective Multicenter Study. 住院期间Vonicog α与血浆来源的血管性血友病因子：一项观察性回顾性多中心研究的结果

IF 4.2 2区医学

Seminars in thrombosis and hemostasis Pub Date : 2026-09-01 Epub Date: 2026-01-13 DOI: 10.1055/a-2779-9176

Valérie Horvais, Catherine Ternisien, Julien Denis-Le-Sève, Philippe Beurrier, Marc Fouassier, Antoine Babuty, Nicolas Drillaud, Brigitte Pan-Petesch, Johann Rose, Vincent Cussac, Sophie Bayart, Benoît Guillet, Marc Trossaërt

{"title":"Vonicog Alfa versus Plasma-Derived von Willebrand Factor during Hospitalization: Results of an Observational Retrospective Multicenter Study.","authors":"Valérie Horvais, Catherine Ternisien, Julien Denis-Le-Sève, Philippe Beurrier, Marc Fouassier, Antoine Babuty, Nicolas Drillaud, Brigitte Pan-Petesch, Johann Rose, Vincent Cussac, Sophie Bayart, Benoît Guillet, Marc Trossaërt","doi":"10.1055/a-2779-9176","DOIUrl":"10.1055/a-2779-9176","url":null,"abstract":"Background: Available products for the treatment of von Willebrand disease (VWD) now include a new recombinant von Willebrand factor (rVWF) in addition to plasma-derived concentrates (pdVWF). However, these two therapies have never been compared directly in either preclinical studies or real-world inpatient settings.Objective: The Hopscotch Will II study examined the treatment of VWD in hospitals and compared the use of pdVWF and rVWF.Methods: Five Rare Bleeding Diseases Centers in Western France retrospectively included patients with VWD over a 48-month period. The data was collected from the BERHLINGO Research Database as well as the French Hospital database, which contains medical information from patient records.Results: Of the 866 patients evaluated in the study, 285 underwent 648 hospitalizations; 126 adult patients (VWD type 3 excluded) were given VWF concentrates during 249 of those hospital stays. rVWF was used in 61% of the cases. The majority of the hospitalizations were motivated by cutaneous-mucosal symptoms in gastroenterology, stomatology, gynecology, and obstetrics. Consumption of rVWF was lower, though the difference in total VWF consumption per stay or per patient per year was not significant: 51 (57)/34 (30) IU/kg/patient/year for pdVWF versus 40 (47)/27 (26) for rVWF (mean [SD]/median [IQR], Wilcoxon's rank sum test, p = 0.2025).Conclusion: rVWF was used in similar patient profiles and for identical procedures, but the cost of treatment with rVWF was significantly lower, regardless of whether or not FVIII was added.","PeriodicalId":21673,"journal":{"name":"Seminars in thrombosis and hemostasis","volume":" ","pages":"723-731"},"PeriodicalIF":4.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13485343/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145966898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Acquired von Willebrand Syndrome: A Comprehensive Review and a Nordic Perspective. 获得性血管性血友病：一个全面的回顾和北欧的观点。

IF 4.2 2区医学

Seminars in thrombosis and hemostasis Pub Date : 2026-09-01 Epub Date: 2025-12-22 DOI: 10.1055/a-2765-9556

Eva Zetterberg, Karin Strandberg, Andreas Hillarp, Vuokko Nummi, Timea Szanto, Anna-Elina Lehtinen, Anne Louise Töllböll Sörensen, Anna Olsson, Ines Vaide, Eva Funding

{"title":"Acquired von Willebrand Syndrome: A Comprehensive Review and a Nordic Perspective.","authors":"Eva Zetterberg, Karin Strandberg, Andreas Hillarp, Vuokko Nummi, Timea Szanto, Anna-Elina Lehtinen, Anne Louise Töllböll Sörensen, Anna Olsson, Ines Vaide, Eva Funding","doi":"10.1055/a-2765-9556","DOIUrl":"10.1055/a-2765-9556","url":null,"abstract":"Abstract: Acquired von Willebrand syndrome (AVWS) is a rare condition characterized by an acquired functional and/or absolute deficiency of the von Willebrand factor (VWF) protein. The absence of widely accepted diagnostic criteria has hampered accurate estimates of incidence and prevalence, which are largely currently unknown. As bleeding symptoms are not included in the most widely used definitions, AVWS should be managed as a risk factor for bleeding, rather than a specific disease entity. The diagnostic workup is cumbersome, involving measurement of both VWF antigen, VWF glycoprotein Ib binding activity, VWF collagen binding activity, and, preferentially, also VWF multimer analyses. Moreover, since the presence of bleeding symptoms is not required for diagnosis, the condition is probably underdiagnosed. In contrast to acquired hemophilia, AVWS is seldom caused by the presence of specific antibodies, but rather secondary to another disorder, most commonly lymphoproliferative, myeloproliferative, cardiovascular, and autoimmune disorders. Pathogenesis of AVWS varies according to the underlying disorder and includes nonspecific adsorption of VWF to antibodies, adsorption onto surfaces of neoplastic cells, mechanical injury, or VWF proteolysis. Treatment includes treating the underlying cause as well as stopping acute bleeds. Here, we present a comprehensive review of what is currently known regarding demographics, diagnostics, and clinical presentation of the syndrome. Since no prospective treatment studies have been performed, treatment choices must be based on data from registries and case reports that are also summarized. Moreover, we present treatment experiences of previously unpublished Nordic cases.","PeriodicalId":21673,"journal":{"name":"Seminars in thrombosis and hemostasis","volume":" ","pages":"628-644"},"PeriodicalIF":4.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145811380","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Platelets in Neonatal Sepsis: Beyond Clotting, from Silent Players to Immune Orchestrators. 血小板在新生儿败血症中的作用：超越凝血，从沉默的参与者到免疫协调者。

IF 4.2 2区医学

Seminars in thrombosis and hemostasis Pub Date : 2026-09-01 Epub Date: 2025-10-27 DOI: 10.1055/a-2731-2399

Rozeta Sokou, Alexandra Lianou, Andreas G Tsantes, Eleni Petrou, Stefanos Bonovas, Georgios Nikolopoulos, Argirios E Tsantes, Nicoletta Iacovidou

引用次数: 0

Beyond Hemolysis and Transfusion: The Untold Story of Blood Groups in Neonatal Disease. 超越溶血和输血：新生儿疾病中不为人知的血型故事。

IF 4.2 2区医学

Seminars in thrombosis and hemostasis Pub Date : 2026-08-31 DOI: 10.1055/a-2943-5876

Rozeta Sokou, Alexia E Palioura, Alexandra Lianou, Stavroula Aggelopoulou, Andreas G Tsantes, Stefanos Bonovas, Vasiliki Mougiou, Zoe Ilidromiti, Theodora Boutsikou, Argirios E Tsantes, Nicoletta Iacovidou

{"title":"Beyond Hemolysis and Transfusion: The Untold Story of Blood Groups in Neonatal Disease.","authors":"Rozeta Sokou, Alexia E Palioura, Alexandra Lianou, Stavroula Aggelopoulou, Andreas G Tsantes, Stefanos Bonovas, Vasiliki Mougiou, Zoe Ilidromiti, Theodora Boutsikou, Argirios E Tsantes, Nicoletta Iacovidou","doi":"10.1055/a-2943-5876","DOIUrl":"https://doi.org/10.1055/a-2943-5876","url":null,"abstract":"Abstract: Blood, as a genetically determined phenotypic characteristic of red blood cells (RBCs), has traditionally been associated with transfusion safety and prevention of hemolytic reactions. However, recent data suggest that the ABO system may influence the predisposition to and the course of various pathological conditions, with potential implications for the development of comorbidities and clinical management of patients. Blood group antigens are expressed on RBCs, platelets, epithelial cells, and endothelial cells, and are involved in fundamental biological functions, such as cell recognition, intercellular adhesion, regulation of hemostasis, and immune response. Many epidemiological studies in adult populations demonstrate an association between specific blood groups of the ABO system and the Rh factor, and a differential risk of cardiovascular, metabolic, and infectious diseases, as well as the development of malignancies; however, in neonates, relevant studies are limited, and the impact of blood group on morbidity during the perinatal period remains unclear. The purpose of this narrative review is to investigate the association between neonatal blood group and the occurrence and severity of critical neonatal comorbidities, through an evaluation of the available clinical and epidemiological evidence, and to clarify the suspected pathogenetic mechanisms, with the aim of supporting future research efforts and improving the clinical care of this vulnerable population.","PeriodicalId":21673,"journal":{"name":"Seminars in thrombosis and hemostasis","volume":" ","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148866602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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 Evolving Knowledge on Primary Hemostasis in Neonates. 新生儿原发性止血知识的发展。

IF 4.2 2区医学

Seminars in thrombosis and hemostasis Pub Date : 2026-08-25 DOI: 10.1055/a-2931-3122

Lorenzo Zanetto, Elena Campello, Mariella Magarotto, Stefano Sartori, Daniela Regazzo, Maurizio Muraca, Eugenio Baraldi, Paolo Simioni

{"title":"The Evolving Knowledge on Primary Hemostasis in Neonates.","authors":"Lorenzo Zanetto, Elena Campello, Mariella Magarotto, Stefano Sartori, Daniela Regazzo, Maurizio Muraca, Eugenio Baraldi, Paolo Simioni","doi":"10.1055/a-2931-3122","DOIUrl":"10.1055/a-2931-3122","url":null,"abstract":"Abstract: Neonatologists spend considerable effort with the aim of preventing neonatal bleeding. Traditionally, platelet failure has been widely considered a crucial determinant of neonatal hemorrhage. However, recent research challenges this view and suggests that platelet hyporeactivity may be part of a carefully balanced hemostatic system. This review explores the broader process of primary hemostasis in both term and preterm infants, beyond platelet function alone, integrating evidence from in vitro, laboratory, and point-of-care studies. Overall, studies raise concerns about the possible contribution of platelet dysfunction to an increased bleeding risk, especially in preterm infants. The clinical relevance of these findings remains uncertain, owing to methodological heterogeneity and the limited correlation with meaningful outcomes. Although platelet hyporeactivity has been largely attributed to a developmental impairment, it might also depend on prevailing up-regulated inhibitory pathways. Nevertheless, functional differences in plasma proteins provide enhanced adhesive capacities, supporting platelet-subendothelium interaction, further facilitated by higher hematocrit levels. Molecular explanations for platelet singularities are being uncovered thanks to omics studies. Advanced technologies such as microfluidics are enabling the study of cross-talking endothelium and platelets. Yet, the details of the interactions between platelets, erythrocytes, and white blood cells remain to be elucidated in the setting of developmental hemostasis. Emerging evidence on distinct pathways driving a lower pro-inflammatory tendency of the neonatal milieu might explain the poorer outcomes of those preterm infants receiving multiple transfusions of adult blood products. Awareness of the unique equilibrium of neonatal primary hemostasis calls for further research into the impact of neonatal therapies on hemostatic balance.","PeriodicalId":21673,"journal":{"name":"Seminars in thrombosis and hemostasis","volume":" ","pages":""},"PeriodicalIF":4.2,"publicationDate":"2026-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148707677","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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