Hamostaseologie最新文献

Hemostatic Considerations of New Treatment Options for Hemophilia During Surgery, Infection, and Bleeding in Children and Adolescents. 儿童和青少年手术、感染和出血期间血友病新治疗方案的止血考虑。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-10-07 DOI: 10.1055/a-2957-0168

Ivonne Wieland, Katharina Becker

{"title":"Hemostatic Considerations of New Treatment Options for Hemophilia During Surgery, Infection, and Bleeding in Children and Adolescents.","authors":"Ivonne Wieland, Katharina Becker","doi":"10.1055/a-2957-0168","DOIUrl":"https://doi.org/10.1055/a-2957-0168","url":null,"abstract":"Abstract: In recent years, the therapeutic landscape for hemophilia has changed significantly, with the approval of several innovative agents, some featuring entirely novel mechanisms of action. These advances have substantially improved outcomes and reduced the treatment burden. However, clinical experience with these novel therapies in special situations-such as bleeding, surgery, trauma, and infection-remains limited. Factor replacement therapy has been the standard of care for a long time, with extensive clinical experience. The introduction of non-factor therapies has raised important questions: Is additional treatment required after injury or during surgery? What are the considerations in cases of sepsis or disseminated intravascular coagulation (DIC)? This summary focuses on emicizumab, efanesoctocog alfa, newly approved TFPI inhibitors, nonacog beta pegol, and the extension of indications for PEGylated FVIII preparations to younger age groups. We will therefore also briefly address these developments. The largest part of this manuscript will focus on emicizumab due to its longer approval history and the most extensive data available. Most other medications were licensed in 2024 or later.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149129364","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

Platelet Type Von Willebrand Disease - Diagnostic and Therapeutic Challenges. 血小板型血管性血友病的诊断和治疗挑战。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-28 DOI: 10.1055/a-2940-6179

Rebekka Wiedenhoefer, Doris Boeckelmann, Hannah Glonnegger, Johannes Oldenburg, Barbara Zieger

{"title":"Platelet Type Von Willebrand Disease - Diagnostic and Therapeutic Challenges.","authors":"Rebekka Wiedenhoefer, Doris Boeckelmann, Hannah Glonnegger, Johannes Oldenburg, Barbara Zieger","doi":"10.1055/a-2940-6179","DOIUrl":"https://doi.org/10.1055/a-2940-6179","url":null,"abstract":"Abstract: Platelet-type von Willebrand disease (PT-VWD) is a rare thrombocytopathy caused by gain-of-function variants in GP1BA, leading to increased binding of von Willebrand factor (VWF). Because its phenotype closely resembles VWD type 2B (VWD 2B), misdiagnosis is common, and distinction requires Ristocetin-induced platelet agglutination (RIPA) mixing studies and molecular genetic analysis. Although individual case reports, case series, and earlier summaries exist, an updated and comprehensive synthesis of all published cases has been lacking. We report a 4-year-old boy presenting with easy bruising, prolonged epistaxis, reduced VWF parameters (VWF collagen-binding activity 0.07 U/mL, VWF-antigen 0.33 U/mL), absence of high-molecular-weight multimers, and enhanced RIPA. Genetic testing identified the pathogenic GP1BA variant p.Met255Val, causing PT-VWD. RIPA mixing studies confirmed the diagnosis. In the course of the disease, the patient developed recurrent mucosal bleeding and a hypersensitivity reaction to tranexamic acid. Therefore, desmopressin was used, and he showed partial clinical benefit from desmopressin without developing thrombocytopenia. To contextualize this case, we performed a literature review (PubMed, May 2025) and identified 67 documented patients with PT-VWD. Over 40% were initially misclassified as VWD 2B. p.Gly249Val and p.Met255Val were the most frequent variants. Baseline thrombocytopenia occurred in approximately one-third of cases, and pregnancy-associated thrombocytopenia was common. According to the literature, platelet transfusions were most effective (in case of major hemorrhage or surgical setting); VWF concentrates or desmopressin showed variable efficacy and occasional transient thrombocytopenia. These data demonstrate the diagnostic challenges of PT-VWD and emphasize the importance of clinical case collection to improve therapeutical management.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148852013","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

Diagnosing Immune Thrombocytopenia: An Update. 诊断免疫性血小板减少症：最新进展。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-27 DOI: 10.1055/a-2926-8391

Nina Cooper, Ulrich J Sachs

{"title":"Diagnosing Immune Thrombocytopenia: An Update.","authors":"Nina Cooper, Ulrich J Sachs","doi":"10.1055/a-2926-8391","DOIUrl":"https://doi.org/10.1055/a-2926-8391","url":null,"abstract":"Abstract: Immune thrombocytopenia (ITP) is an acquired autoimmune disorder characterized by increased platelet destruction and impaired platelet production, resulting in isolated thrombocytopenia. Despite advances in understanding disease pathophysiology, diagnosis remains challenging because no single clinical or laboratory test definitively establishes the diagnosis. Current international guidelines therefore continue to define ITP primarily as a diagnosis of exclusion based on clinical assessment, complete blood count, and peripheral blood smear evaluation.Abstract: Recent developments in platelet immunology and laboratory diagnostics have expanded the range of adjunctive diagnostic tools. Glycoprotein-specific platelet autoantibody assays, including monoclonal antibody immobilization of platelet antigens (MAIPA)-based methods, provide high specificity for immune-mediated platelet destruction but remain limited by moderate sensitivity and restricted standardization. Biomarkers reflecting thrombopoiesis, particularly immature platelet fraction, thrombopoietin levels, support differentiation between hyperdestructive and hypoproductive thrombocytopenia, especially when integrated into multivariable diagnostic algorithms.Abstract: Current diagnostic strategies differ across guidelines. Whereas ASH, ISTH-associated initiatives and Onkopedia recommendations emphasize restrained and clinically guided testing, the recent Japanese guideline incorporates thrombopoietin levels and immature platelet fraction into a more laboratory-oriented diagnostic framework. Overall, laboratory investigations should still be regarded as supportive rather than definitive. Future diagnostic strategies are expected to integrate clinical assessment with immunologic, thrombopoietic, and cellular biomarkers, enabling a more pathophysiology-based classification of thrombocytopenic disorders and improving diagnostic precision and individualized patient management.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148842259","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

A novel Mutation p.Thr308Asn in the Snout-like Region of the Fibrinogen Bβ Chain: Analysis of the Pathogenic Mechanism of Hereditary Hypofibrinogenemia. 纤维蛋白原Bβ链鼻状区新突变p.s thr308asn：遗传性低纤维蛋白原血症发病机制分析

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-26 DOI: 10.1055/a-2937-0837

Jinxiong Jiang, Xiaoying Lü, Ziyan Wei, Xiaocheng Luo, Yongxin Yu, Xiufeng Gan, Xiaodan Wang, Lisa Zhao, Zhenrui Li, Yu He, Fuyong Zhang

{"title":"A novel Mutation p.Thr308Asn in the Snout-like Region of the Fibrinogen Bβ Chain: Analysis of the Pathogenic Mechanism of Hereditary Hypofibrinogenemia.","authors":"Jinxiong Jiang, Xiaoying Lü, Ziyan Wei, Xiaocheng Luo, Yongxin Yu, Xiufeng Gan, Xiaodan Wang, Lisa Zhao, Zhenrui Li, Yu He, Fuyong Zhang","doi":"10.1055/a-2937-0837","DOIUrl":"10.1055/a-2937-0837","url":null,"abstract":"Abstract: BACKGROUND: Fibrinogen, encoded by FGA, FGB, and FGG, is essential for blood coagulation. Mutations in these genes cause congenital fibrinogen disorders, leading to bleeding and thrombotic complications. The Bβ chain is the rate-limiting component in fibrinogen production, and its C-terminal region is essential for assembly and secretion. However, the pathogenic mechanism of missense mutations in the FGB gene remains to be fully elucidated.Abstract: OBJECTIVE: This study aimed to analyze the genotype and phenotype of a pedigree with hereditary hypofibrinogenemia and to preliminarily elucidate the potential pathogenic mechanism.Abstract: METHODS: Fibrinogen levels were measured using the Clauss and PT-derived methods. DNA sequence analysis was performed to identify the mutation. Conservation and protein structural modeling analyses were performed using online bioinformatics tools. The pathogenicity of the variant was assessed according to the ACMG/AMP guidelines.Abstract: RESULTS: The fibrinogen level of the proband was 0.74 g/L. Thromboelastography (TEG) showed that the proband's clot kinetics time (K time) was 4.4 min (prolonged), thrombus generation angle (α angle) was 42.6° (decreased), and clotting index (CI) was -4.8 (decreased). Genetic analysis revealed a heterozygous c.923C > A (p.Thr308Asn) mutation in FGB gene. Bioinformatics and modeling analysis suggested that the missense mutation may have a deleterious effect on fibrinogen. The variant was classified as likely pathogenic based on the ACMG/AMP guidelines.Abstract: CONCLUSION: The c.923C > A(p.Thr308Asn) mutation in exon 6 of the FGB gene may be responsible for the reduced fibrinogen level observed in this pedigree. To our knowledge, this point mutation is reported for the first time.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148834771","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

Factor XI Inhibition: Adult Evidence and Paediatric Considerations. 因子XI抑制：成人证据和儿科考虑。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-07 DOI: 10.1055/a-2918-8066

Susanne Holzhauer, Christoph Male

引用次数: 0

Performance Evaluation of Different FIX Activity Assays for Determining Nonacog Beta Pegol (N9-GP, Refixia) Activity Using Reagent-/Platform-Specific Conversion Factors. 使用试剂/平台特定转换因子测定Nonacog Beta Pegol （N9-GP, Refixia®）活性的不同FIX活性测定法的性能评估

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-01 Epub Date: 2025-08-29 DOI: 10.1055/a-2599-9908

Christian Irsara, Andrea Griesmacher, Bernhard Strasser, Axel Schlagenhauf, Christina Sorschag, Erich Wimmer, Florian Prüller, Janne Cadamuro, Johannes Radek, Josef Tomasits, Josef Seier, Peter Fraunberger, Peter Quehenberger, Gerald Lirk, Sabine Sussitz-Rack, Sylvia Mink, Alexander Haushofer

{"title":"Performance Evaluation of Different FIX Activity Assays for Determining Nonacog Beta Pegol (N9-GP, Refixia) Activity Using Reagent-/Platform-Specific Conversion Factors.","authors":"Christian Irsara, Andrea Griesmacher, Bernhard Strasser, Axel Schlagenhauf, Christina Sorschag, Erich Wimmer, Florian Prüller, Janne Cadamuro, Johannes Radek, Josef Tomasits, Josef Seier, Peter Fraunberger, Peter Quehenberger, Gerald Lirk, Sabine Sussitz-Rack, Sylvia Mink, Alexander Haushofer","doi":"10.1055/a-2599-9908","DOIUrl":"10.1055/a-2599-9908","url":null,"abstract":"Background: Nonacog beta pegol (N9-GP) is a glycoPEGylated FIX replacement product with extended half-life for treatment of haemophilia B patients. Monitoring of N9-GP with clotting-based one-stage FIX assays is complicated by high variations, mainly due to reagent-specific interference with polyethylene glycol.Methods: In 11 distinct specialized coagulation laboratories in Austria, N9-GP spiked samples were measured in replicates in two distinct surveys, 3 years apart, using five different one-stage assay reagents and one chromogenic FIX assay. Regression analysis was used to investigate if back-calculation of N9-GP levels is feasible.Results: We could demonstrate a linear relationship between the spiked N9-GP concentration and measured FIX activity levels for all examined assays, suggesting that N9-GP activity may be back-calculated using reagent-/platform-specific conversion factors. Within-laboratory variation after 3 years was acceptable in most, but not all, laboratories.Conclusion: We demonstrate that back-calculation of N9-GP activity levels may be possible when using one-stage FIX assays. However, we recommend that every laboratory ascertain its own conversion factor. When measuring real patient samples, we encourage simultaneous measurement of N9-GP spiked control material with known concentrations to ensure the validity of the current back-calculation.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":"283-292"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144979005","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

Correction: A novel Mutation p.Thr308Asn in the Snout-like Region of the Fibrinogen Bβ Chain: Analysis of the Pathogenic Mechanism of Hereditary Hypofibrinogenemia. 修正：纤维蛋白原Bβ链鼻状区域的新突变p.s thr308asn：遗传性低纤维蛋白原血症的致病机制分析。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-01 Epub Date: 2026-10-07 DOI: 10.1055/a-2967-9687

Jinxiong Jiang, Xiaoying Lü, Ziyan Wei, Xiaocheng Luo, Yongxin Yu, Xiufeng Gan, Xiaodan Wang, Lisa Zhao, Zhenrui Li, Yu He, Fuyong Zhang

引用次数: 0

Antithrombotic Therapy in Cancer Patients with Cardiovascular Diseases: Daily Practice Recommendations by the Hemostasis Working Party of the German Society of Hematology and Medical Oncology (DGHO) and the Society for Thrombosis and Hemostasis Research (GTH e.V.). 心血管疾病癌症患者的抗血栓治疗：德国血液学与肿瘤内科学会 (DGHO) 和血栓与止血研究学会 (GTH e.V.) 的止血工作组提出的日常实践建议。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-01 Epub Date: 2024-07-15 DOI: 10.1055/a-2337-4025

Stefani Parmentier, Steffen Koschmieder, Larissa Henze, Martin Griesshammer, Axel Matzdorff, Tamam Bakchoul, Florian Langer, Rosa Sonja Alesci, Daniel Duerschmied, Goetz Thomalla, Hanno Riess

{"title":"Antithrombotic Therapy in Cancer Patients with Cardiovascular Diseases: Daily Practice Recommendations by the Hemostasis Working Party of the German Society of Hematology and Medical Oncology (DGHO) and the Society for Thrombosis and Hemostasis Research (GTH e.V.).","authors":"Stefani Parmentier, Steffen Koschmieder, Larissa Henze, Martin Griesshammer, Axel Matzdorff, Tamam Bakchoul, Florian Langer, Rosa Sonja Alesci, Daniel Duerschmied, Goetz Thomalla, Hanno Riess","doi":"10.1055/a-2337-4025","DOIUrl":"10.1055/a-2337-4025","url":null,"abstract":"Abstract: Active cancer by itself but also chemotherapy is associated with an increased risk of cardiovascular disease (CVD) and especially coronary artery disease (CAD) and atrial fibrillation (AF). The frequency of CVD, CAD, and AF varies depending on comorbidities (particularly in older patients), cancer type, and stage, as well as the anticancer therapeutic being taken. Many reports exist for anticancer drugs being associated with CVD, CAD, and AF, but robust data are often lacking. Because of this, each patient needs an individual structured approach concerning thromboembolic and bleeding risk, drug-drug interactions, as well as patient preferences to evaluate the need for anticoagulation therapy and targeting optimal symptom control. Interruption of specific cancer therapy should be avoided to reduce the potential risk of cancer progression. Nevertheless, additional factors like thrombocytopenia and anticoagulation in the elderly and frail patient with cancer cause additional challenges which need to be addressed in daily clinical management. Therefore, the aim of these recommendations is to summarize the available scientific data on antithrombotic therapy (both antiplatelet and anticoagulant therapy) in cancer patients with CVD and in cases of missing data providing guidance for optimal careful decision-making in daily routine.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":"250-271"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141621841","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

Sicherheit von Prothrombinkomplex-Konzentrat bei Notfalloperationen in Abhängigkeit vom Spiegel oraler Faktor-Xa-Inhibitoren. 在口服因子Xa抑制剂的紧急手术中凝血酶原复合物浓度的安全性。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-01 Epub Date: 2026-08-21 DOI: 10.1055/a-2902-5143

引用次数: 0

Leitlinie der Gesellschaft für Thrombose- und Hämostaseforschung (GTH) zur Struktur- und Prozessqualität von Hämophilie-Zentren. 血栓和止血研究协会（GTH）关于血友病中心结构和过程质量的指南。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-01 Epub Date: 2024-12-04 DOI: 10.1055/a-2410-8557

H Eichler, M Albisetti, S Halimeh, R Knöfler, C Königs, F Langer, W Miesbach, J Oldenburg, U Scholz, W Streif, R Klamroth

引用次数: 0