Hamostaseologie最新文献_第3页

Structural Analysis of von Willebrand Factor. von Willebrand因子的结构分析。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-02-01 Epub Date: 2026-02-17 DOI: 10.1055/a-2751-7066

Maria A Brehm

引用次数: 0

Von Willebrand Factor at the Crossroads of Hemostasis and Inflammation. 血管性血友病因子在止血和炎症的十字路口。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-02-01 Epub Date: 2026-02-17 DOI: 10.1055/a-2755-5477

Hamideh Yadegari

{"title":"Von Willebrand Factor at the Crossroads of Hemostasis and Inflammation.","authors":"Hamideh Yadegari","doi":"10.1055/a-2755-5477","DOIUrl":"https://doi.org/10.1055/a-2755-5477","url":null,"abstract":"Von Willebrand factor (VWF) is a large multimeric glycoprotein critical for hemostasis, mediating platelet adhesion to injured vessels and stabilizing circulating factor VIII. However, accumulating evidence reveals a complex, context-dependent role for VWF in inflammation and innate immunity that extends well beyond coagulation. VWF acts not only as a biomarker of endothelial activation but also as an active participant in immune responses. VWF directly interacts with major immune cell types-including macrophages, polymorphonuclear leukocytes (neutrophils), and dendritic cells-through both its endothelial-anchored and plasma forms. VWF facilitates leukocyte recruitment and transmigration across the vessel wall, while its interactions also promote macrophage and neutrophil activation as well as NET formation. VWF's immunomodulatory functions are further highlighted by its binding to extracellular DNA, smooth muscle cells, complement components (C1q and C3), and bacterial pathogens under flow conditions. Furthermore, VWF indirectly influences inflammation via its crucial role in Weibel-Palade body formation, a process that co-packages vital inflammatory mediators like P-selectin and angiopoietin-2. Markedly elevated VWF levels are consistently observed across acute and chronic inflammatory conditions such as sepsis, COVID-19, and autoimmune disorders, confirming its relevance as both a diagnostic marker and a therapeutic target. A comprehensive understanding of VWF's diverse functions in vascular inflammation is crucial for developing targeted therapeutics-including nanobodies, ADAMTS13 variants, and VWF interaction inhibitors-capable of modulating pathological thrombo-inflammation while preserving physiological hemostasis.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"46 1","pages":"34-43"},"PeriodicalIF":2.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146215071","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

Primäre Immunthrombozytopenie: Erhöhtes Krebsrisiko für Erwachsene. “原发性免疫血小板减少：成人癌症风险增加”。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-02-01 Epub Date: 2026-02-17 DOI: 10.1055/s-0046-1817055

引用次数: 0

[Digitalisierung im Gesundheitswesen - eine kritische Bestandsaufnahme]. [卫生保健数字化——关键评估]。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-02-01 Epub Date: 2026-02-17 DOI: 10.1055/a-2661-6084

Christoph Sucker, Jürgen Koscielny

引用次数: 0

Correction: Oxidative Stress, NADPH Oxidases, and Arteries. 更正：氧化应激、NADPH氧化酶和动脉。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-02-01 Epub Date: 2026-04-23 DOI: 10.1055/a-2839-5580

Qi-An Sun, Marschall S Runge, Nageswara R Madanchi

引用次数: 0

Die Gesellschaft für Thrombose- und Hämostaseforschung e.V. informiert. 血栓和止血研究协会。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-02-01 Epub Date: 2026-02-17 DOI: 10.1055/a-2661-6223

引用次数: 0

Management of Splanchnic Venous Thrombosis: An Update. 内脏静脉血栓形成的处理：最新进展。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-02-01 Epub Date: 2026-02-17 DOI: 10.1055/a-2749-6835

Heiko Rühl, Sara Reda

{"title":"Management of Splanchnic Venous Thrombosis: An Update.","authors":"Heiko Rühl, Sara Reda","doi":"10.1055/a-2749-6835","DOIUrl":"10.1055/a-2749-6835","url":null,"abstract":"This review provides an updated and comprehensive overview of the diagnosis and management of splanchnic venous thrombosis (SVT), a rare but clinically important manifestation of venous thrombosis involving the portal, mesenteric, splenic, and hepatic veins. The aim of this article is to summarize and critically appraise recent advances in the evidence base for anticoagulant therapy, prognosis, and clinical decision-making across the diverse clinical settings in which SVT occurs, including the most important subgroups of liver disease, myeloproliferative neoplasms, and solid malignancy. The review integrates findings from prospective studies, meta-analyses, and registry data and highlights the evolving role of direct oral anticoagulants (DOACs) as an alternative to vitamin K antagonists and low-molecular-weight heparins. Meta-analyses demonstrate that anticoagulation increases the likelihood of vessel recanalization and improves survival in cirrhotic portal vein thrombosis (PVT) without increasing major bleeding. Pooled data suggest that DOACs achieve at least comparable efficacy to conventional anticoagulant drugs, with lower rates of thrombus growth and major bleeding, while low-dose rivaroxaban has proven effective for secondary prevention in chronic non-cirrhotic PVT. In cancer-associated SVT, anticoagulation reduces recurrence with acceptable bleeding risk, and in Budd-Chiari syndrome, DOACs show promising safety and efficacy in selected patients. Despite major progress in recent years, most evidence still remains observational, and randomized trials, pediatric data, and studies on long-term secondary prevention are urgently needed to further refine and standardize SVT management.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"46 1","pages":"71-77"},"PeriodicalIF":2.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146214972","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

Bei HIT sind sequenzielle Immunoassays sinnvoll. 在HIT中，序列免疫试验是有用的。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-02-01 Epub Date: 2026-02-17 DOI: 10.1055/s-0046-1817056

引用次数: 0

Gene Therapy of Haemophilia: Current Status and Future Directions. 血友病的基因治疗：现状与未来方向。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-02-01 Epub Date: 2026-02-17 DOI: 10.1055/a-2751-7625

Wolfgang Miesbach

{"title":"Gene Therapy of Haemophilia: Current Status and Future Directions.","authors":"Wolfgang Miesbach","doi":"10.1055/a-2751-7625","DOIUrl":"https://doi.org/10.1055/a-2751-7625","url":null,"abstract":"Gene therapy represents a paradigm shift in haemophilia management, offering the potential for sustained factor expression and freedom from prophylactic infusions. Two adeno-associated virus (AAV)-based gene therapies are now approved. Long-term follow-up data demonstrate remarkable durability, with 13-year results showing sustained reductions in annualized bleeding rates in patients with haemophilia B. Outcomes differ between haemophilia A and B: factor VIII levels in haemophilia A decline after peaking, whereas factor IX expression in haemophilia B remains more stable. Recent analyses confirm significant reductions in bleeding rates and treatment requirements, along with well-characterized safety profiles. Liver toxicity remains the primary safety concern, with transaminase elevations typically responding to corticosteroids. Rare adverse events include one reported case of inhibitor development and very few thrombotic events. Reported malignancies to date have not been associated with gene therapy. Current limitations include eligibility restrictions due to preexisting neutralizing antibodies, immune responses to AAV capsids, and variable patient outcomes. These challenges may contribute to slower adoption despite regulatory approval. Emerging approaches such as CRISPR-Cas9 gene editing, high-active factor variants, and novel delivery systems are under investigation. Key implementation issues include outcome-based reimbursement, hub-and-spoke treatment models, and ensuring equitable global access.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"46 1","pages":"10-16"},"PeriodicalIF":2.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146215028","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

Thrombin Generation Assays: Possibilities and Limitations. 凝血酶生成测定：可能性和局限性。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-02-01 Epub Date: 2026-02-17 DOI: 10.1055/a-2753-9825

Behnaz Pezeshkpoor, Johannes Oldenburg, Jens Müller

{"title":"Thrombin Generation Assays: Possibilities and Limitations.","authors":"Behnaz Pezeshkpoor, Johannes Oldenburg, Jens Müller","doi":"10.1055/a-2753-9825","DOIUrl":"https://doi.org/10.1055/a-2753-9825","url":null,"abstract":"Thrombin generation assays (TGA) provide a dynamic and integrative assessment of thrombin generation in clotting plasma ex vivo. The method is characterized by triggering coagulation, typically via the extrinsic pathway, and continuously measuring thrombin activity using a fluorogenic peptide substrate to derive key parameters such as peak thrombin and the endogenous thrombin potential. Several assay platforms are currently available, with the original Calibrated Automated Thrombogram (CAT) still being widely used in clinical and research settings, not least due to its flexibility and ability to analyze both platelet-poor as well as platelet-rich plasma. Thrombin generation assays have the potential to support the evaluation and monitoring of treatment of bleeding disorders, including hemophilia A and B and other inherited or acquired coagulation factor deficiencies. They may contribute to risk stratification in thrombotic disorders, and support the assessment of anticoagulant therapies. However, besides ongoing developments and optimization of trigger reagents, inter-assay variability and susceptibility to pre- and analytical variables challenge assay standardization and inter-laboratory comparability. Continued refinement, harmonization, and prospective clinical validation will be essential to unlock the full diagnostic potential of TGA.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"46 1","pages":"65-70"},"PeriodicalIF":2.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146215117","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