HamostaseologiePub Date : 2026-02-01Epub Date: 2026-02-17DOI: 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":"<p><p>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.</p>","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}
HamostaseologiePub Date : 2026-02-01Epub Date: 2026-02-17DOI: 10.1055/a-2661-6223
{"title":"Die Gesellschaft für Thrombose- und Hämostaseforschung e.V. informiert.","authors":"","doi":"10.1055/a-2661-6223","DOIUrl":"https://doi.org/10.1055/a-2661-6223","url":null,"abstract":"","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"46 1","pages":"78-80"},"PeriodicalIF":2.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146214998","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}
HamostaseologiePub Date : 2026-02-01Epub Date: 2026-02-17DOI: 10.1055/s-0046-1817056
{"title":"Bei HIT sind sequenzielle Immunoassays sinnvoll.","authors":"","doi":"10.1055/s-0046-1817056","DOIUrl":"10.1055/s-0046-1817056","url":null,"abstract":"","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"46 1","pages":"8"},"PeriodicalIF":2.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146214938","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}
HamostaseologiePub Date : 2026-02-01Epub Date: 2026-02-17DOI: 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":"10.1055/a-2753-9825","url":null,"abstract":"<p><p>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.</p>","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}
HamostaseologiePub Date : 2026-02-01Epub Date: 2026-02-17DOI: 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":"10.1055/a-2751-7625","url":null,"abstract":"<p><p>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.</p>","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}
{"title":"Immune Response Mechanisms in Haemophilia A.","authors":"Amina Abdelmageed, Clivia Lisowski, Janine Becker-Gotot","doi":"10.1055/a-2748-8775","DOIUrl":"10.1055/a-2748-8775","url":null,"abstract":"<p><p>Haemophilia A (HA) is an X-linked bleeding disorder caused by the deficiency or dysfunction of coagulation factor VIII (FVIII). Substitution therapy with recombinant or plasma-derived FVIII effectively restores haemostasis but carries the risk of eliciting FVIII-specific neutralizing antibodies known as inhibitors. Inhibitor formation reflects the absence of central immune tolerance, driven by complex interactions among antigen-presenting cells, B cells and CD4 T cell subsets. Recent studies highlight the essential roles of T cell polarization, costimulatory signalling and cytokine networks in shaping the immunogenic or tolerogenic outcome of FVIII exposure. This review summarizes mechanistic insights into how distinct CD4 T cell subpopulations, including Th1, Th2, Th17, follicular helper (Tfh), follicular regulatory (Tfr) and regulatory T cells (Tregs), influence inhibitor development in HA. Furthermore, it discusses emerging immunological concepts and cellular pathways that could be targeted to achieve durable tolerance towards FVIII, with emphasis on translational strategies that align mechanistic understanding with clinical need.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"46 1","pages":"17-23"},"PeriodicalIF":2.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146214985","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}
HamostaseologiePub Date : 2026-02-01Epub Date: 2026-02-19DOI: 10.1055/a-2749-2763
Tobias Feige, Agnes Bosbach, Kim J Krott, Margitta Elvers
{"title":"Role of Platelets in Abdominal Aortic Aneurysm Formation and Progression: New Aspects from Experimental and Clinical Approaches.","authors":"Tobias Feige, Agnes Bosbach, Kim J Krott, Margitta Elvers","doi":"10.1055/a-2749-2763","DOIUrl":"10.1055/a-2749-2763","url":null,"abstract":"<p><p>Abdominal aortic aneurysm (AAA) is a vascular disease with an atherosclerotic background resulting in progressive abdominal aortic dilation. The mechanisms of AAA progression include chronic inflammation within the aneurysm segment, reinforcing extracellular matrix degradation, and weakening of the aortic vessel wall. Platelets are essential mediators of hemostasis and play a dominant role in cardiovascular disease. Recent data suggest a pivotal role of platelets in AAA formation and progression by dysregulated platelet activation. These findings include a potential crosstalk of platelets with different cell types such as macrophages and fibroblasts, which amplifies the inflammatory response within the aortic tissue and promotes aortic wall stiffening. Experimental studies provided evidence for platelets to directly contribute to AAA formation and progression via the collagen receptor glycoprotein VI by promoting vascular inflammation, as well as aortic wall remodeling. Moreover, platelet and red blood cell interactions via the TSP-1-CD36 axis reinforces aneurysm formation via elevated procoagulant activity of both cells in experimental mice and AAA patients. Therefore, platelets and a platelet-mediated cellular crosstalk play a crucial role in AAA by promoting the development and progression of AAA. To date, no therapeutic treatment is available and surgical repair is the only option to prevent patients from aortic rupture. Thus, there is a strong need for an effective therapy to attenuate AAA progression. This review highlights the mechanisms of platelet activation and the relevance for the identification of new platelet-derived targets to develop a drug-based therapy for AAA patients.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"46 1","pages":"53-64"},"PeriodicalIF":2.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146229936","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}
HamostaseologiePub Date : 2026-02-01Epub Date: 2026-02-17DOI: 10.1055/a-2755-7600
Friedrich Reusswig, Carsten Deppermann
{"title":"The Role of Platelets Beyond Hemostasis.","authors":"Friedrich Reusswig, Carsten Deppermann","doi":"10.1055/a-2755-7600","DOIUrl":"10.1055/a-2755-7600","url":null,"abstract":"<p><p>Platelets, small anucleate cells derived from megakaryocytes, are key drivers of hemostasis and thrombosis. There is accumulating evidence, however, that platelets play roles beyond thrombosis and hemostasis, especially in inflammation, infection, and tissue regeneration. They can recruit, stimulate, and modulate immune cells by direct interaction or via the release of soluble factors. During inflammation, platelets maintain vascular integrity in the lung, skin, and brain through interactions of their GPVI, CLEC-2, and GPIb receptors as well as factors released from α-granules and dense granules. They cooperate with monocytes and macrophages to fight bacteria in the bloodstream and in the liver where they accumulate on Kupffer cell-bacteria complexes to support pathogen destruction. Upon liver damage, platelets adhere to the liver sinusoids and release bioactive molecules like serotonin and fibrinogen to support reparative processes. Here, we provide a review of some of the multiple roles of platelets beyond their classical role in hemostasis.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"46 1","pages":"44-52"},"PeriodicalIF":2.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146215077","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}