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Management of Young and Ageing Women with Afibrinogenemia and Hypofibrinogenemia. 年轻和老年妇女纤维蛋白原血症和低纤维蛋白原血症的管理。
IF 2.7 4区 医学
Hamostaseologie Pub Date : 2025-05-27 DOI: 10.1055/a-2568-1255
Alessandro Casini, Philippe de Moerloose
{"title":"Management of Young and Ageing Women with Afibrinogenemia and Hypofibrinogenemia.","authors":"Alessandro Casini, Philippe de Moerloose","doi":"10.1055/a-2568-1255","DOIUrl":"https://doi.org/10.1055/a-2568-1255","url":null,"abstract":"<p><p>Congenital afibrinogenemia and hypofibrinogenemia are rare hereditary coagulation disorders characterized by the absence or deficiency of fibrinogen. These conditions pose unique challenges for women across their lifespan, including heavy menstrual bleeding (HMB), hemorrhagic ovarian cysts, complications during pregnancy and the postpartum period, as well as bleeding occurring later in life. HMB is frequent and adversely impacts quality of life, often necessitating hormonal therapy, antifibrinolytics, or fibrinogen replacement. Hemorrhagic ovarian cysts can result in life-threatening hemoperitoneum, requiring prompt intervention to manage bleeding and preserve ovarian function. Pregnancy in women with severe fibrinogen deficiencies carries a high risk of miscarriage, placental abruption, and postpartum hemorrhage. Multidisciplinary care, fibrinogen replacement, and vigilant monitoring are crucial to optimize maternal and fetal outcomes. Although understudied in this population, bleeding can occur later in their life, especially due to the increased incidence of gynecological pathologies. Tailored management strategies, including hormonal and surgical interventions, are essential. Despite recent advances in our understanding of these conditions, significant knowledge gaps persist regarding the prevalence, risk factors, and optimal management of specific complications. This review synthesizes current findings and provides practical recommendations to guide the care of young and ageing women with afibrinogenemia and hypofibrinogenemia. Further research is needed to refine treatment protocols and improve outcomes for this vulnerable population.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144163961","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
Evidence in Haemophilia Assessment: The Haemophilia Joint Health Score. 血友病评估的证据：血友病关节健康评分。
IF 2.7 4区 医学
Hamostaseologie Pub Date : 2025-05-23 DOI: 10.1055/a-2575-1475
Lukas Graf
{"title":"Evidence in Haemophilia Assessment: The Haemophilia Joint Health Score.","authors":"Lukas Graf","doi":"10.1055/a-2575-1475","DOIUrl":"https://doi.org/10.1055/a-2575-1475","url":null,"abstract":"<p><p>Haemophilia is a rare genetic bleeding disorder that primarily affects males and results in the deficiency of clotting factors VIII (haemophilia A) or IX (haemophilia B). One of the most debilitating long-term complications of haemophilia is chronic joint damage with pain, and reduced mobility, due to bleeding into the joints. As the primary cause of morbidity in people with haemophilia (PwH), joint health assessment is critical for disease management and optimizing patient outcomes. Among the tools developed to monitor joint health in PwH, the Haemophilia Joint Health Score (HJHS) has emerged as the most widely used and validated clinical tool. There is evidence supporting the use of the HJHS in both children and adults. In contrast to scoring methods that incorporate imaging techniques, which primarily describe the morphology of the joints, the HJHS allows for the assessment and monitoring of joint functionality.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144133164","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
Investigation of Bleeding Disorders: When and How Should We Test Platelet Functions? 出血性疾病的调查：何时以及如何检测血小板功能？
IF 2.7 4区 医学
Hamostaseologie Pub Date : 2025-05-12 DOI: 10.1055/a-2535-9137
Paolo Gresele, Emanuela Falcinelli, Loredana Bury
{"title":"Investigation of Bleeding Disorders: When and How Should We Test Platelet Functions?","authors":"Paolo Gresele, Emanuela Falcinelli, Loredana Bury","doi":"10.1055/a-2535-9137","DOIUrl":"https://doi.org/10.1055/a-2535-9137","url":null,"abstract":"<p><p>Inherited platelet disorders (IPDs) are rare conditions with diverse underlying pathophysiology which should be suspected in patients presenting with mucocutaneous bleeding or hemorrhages upon hemostatic challenges, in the presence or not of thrombocytopenia. Identifying IPDs is critical for providing appropriate care, preventing misdiagnosis, and avoiding unnecessary interventions, such as splenectomy. Syndromic IPDs, which may be associated with severe complications like kidney failure, infection, and malignancies, underscore the importance of accurate diagnosis and tailored management.Diagnosing IPDs remains challenging, requiring a comprehensive approach that integrates clinical assessment, evaluation of the bleeding history using standardized tools, like the ISTH-BAT, and first-line laboratory tests, such as light transmission aggregometry and flow cytometry. Second-line and specialized tests, including transmission electron microscopy, genetic analysis, and biochemical studies, may provide further insight in complex cases. Technological advancements, including multicolor flow cytometry and microfluidic tools, may in perspective improve IPD diagnostics by providing high-throughput and precise laboratory assays. In particular, mass cytometry and multi-omics may contribute to unraveling IPD pathophysiology, identifying novel markers, and refining disease classification. The application of artificial intelligence shows potential for improving diagnostic accuracy through the automated analysis of platelet morphology and function, from flow cytometry and digital microscopy assays, and for improving the understanding of pathogenic mechanisms of IPD through the examination of big data.This review summarizes current IPD platelet function testing strategies, emphasizing the need for a structured, tiered approach and examining emerging technologies and AI applications that could revolutionize diagnostic workflows, leading to personalized care and to an expanded understanding of IPDs.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144062888","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
Atrial Fibrillation in a Patient with Hemophilia A: A Case Report and Therapeutic Challenges. a型血友病患者心房颤动：一例报告和治疗挑战。
IF 2.7 4区 医学
Hamostaseologie Pub Date : 2025-05-06 DOI: 10.1055/a-2496-1491
Karina Althaus, Jürgen Schreieck, Günalp Uzun, Stefanie Hammer, Tamam Bakchoul
{"title":"Atrial Fibrillation in a Patient with Hemophilia A: A Case Report and Therapeutic Challenges.","authors":"Karina Althaus, Jürgen Schreieck, Günalp Uzun, Stefanie Hammer, Tamam Bakchoul","doi":"10.1055/a-2496-1491","DOIUrl":"https://doi.org/10.1055/a-2496-1491","url":null,"abstract":"<p><p>The increasing age of patients with congenital hemophilia poses new challenges for clinicians. Not only the consequences of bleeding but also age-related diseases, especially cardiovascular disorders, are of great concern. Factors such as hypertension, diabetes, or hyperlipidemia further increase the cardiovascular risk in elderly patients with hemophilia. Preventive treatment of these cardiovascular diseases may therefore also be necessary in hemophilia patients. However, determining the optimal level of anticoagulation in patients with reduced levels of coagulation factors is often difficult and requires an individualized approach. Given the absence of substantial evidence from large clinical trials, clinicians rely on case reports and expert opinions to guide the therapy. This case report details the individual risk-benefit assessment and management of a 57-year-old hemophilia A patient with atrial fibrillation.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144060705","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
Thromboprophylaxis in Children: Navigating Uncharted Waters. 儿童血栓预防：导航未知水域。
IF 2.7 4区 医学
Hamostaseologie Pub Date : 2025-04-27 DOI: 10.1055/a-2511-3364
Cornelia Heleen van Ommen
{"title":"Thromboprophylaxis in Children: Navigating Uncharted Waters.","authors":"Cornelia Heleen van Ommen","doi":"10.1055/a-2511-3364","DOIUrl":"https://doi.org/10.1055/a-2511-3364","url":null,"abstract":"<p><p>The rising incidence of pediatric thrombosis, increasing nearly 10% annually over the past decade, underlines a need for effective preventive strategies, particularly in hospitalized children. This narrative review explores existing literature on venous thromboembolism prevention in pediatric patients to clarify current strategies in children undergoing general, orthopaedic, and Fontan surgeries, and with conditions such as acute lymphoblastic leukemia and gastrointestinal disease. While recent studies have provided some insights into potential preventive strategies, real evidence is lacking on the best practices for thromboprophylaxis in these patient groups. In the absence of substantial studies, clinicians must rely on individual assessments weighting thrombotic and bleeding risk to effectively and safely manage these \"uncharted waters.\"</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144041330","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
Fibrinogen Replacement: A Questionable Dogma. 纤维蛋白原替代：一个有问题的教条。
IF 2.7 4区 医学
Hamostaseologie Pub Date : 2025-04-27 DOI: 10.1055/a-2535-8910
Patricia Duque, Wolfgang Korte
{"title":"Fibrinogen Replacement: A Questionable Dogma.","authors":"Patricia Duque, Wolfgang Korte","doi":"10.1055/a-2535-8910","DOIUrl":"https://doi.org/10.1055/a-2535-8910","url":null,"abstract":"<p><p>Management of hemostasis in the perioperative setting, in trauma or in acute care, has considerably changed over the last two decades. Viscoelastic testing and single-factor replacement therapies have become cornerstones of the respective clinical approaches. Here, we illuminate the basic theories for these approaches as well as the important evidence available. Both viscoelastic assays and single-factor replacements are important improvements; their use must be based on the strongest scientific evidence available.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144027743","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
Accelerated Fibrinolysis: A Tendency to Bleed? 加速纤维蛋白溶解：出血倾向？
IF 2.7 4区 医学
Hamostaseologie Pub Date : 2025-04-27 DOI: 10.1055/a-2561-6786
Dino Mehic, Ingrid Pabinger, Johanna Gebhart
{"title":"Accelerated Fibrinolysis: A Tendency to Bleed?","authors":"Dino Mehic, Ingrid Pabinger, Johanna Gebhart","doi":"10.1055/a-2561-6786","DOIUrl":"https://doi.org/10.1055/a-2561-6786","url":null,"abstract":"<p><p>Hyperfibrinolysis is rarely investigated as an underlying mechanism in patients with mild-to-moderate bleeding disorders (MBDs) and bleeding disorders of unknown cause (BDUC). Hereditary hyperfibrinolytic disorders, including α2-antiplasmin (α2-AP) deficiency, plasminogen activator inhibitor type 1 (PAI-1) deficiency, Quebec platelet disorder, and tissue plasminogen activator (tPA) excess, present with mild-to-moderate bleeding symptoms that are common in patients with MBD or BDUC, but may also manifest as life-threatening bleeding. This review summarizes the available data on hyperfibrinolysis in MBD and BDUC patients, and its assessment by various methods such as measurement of fibrinolytic factors, global hemostatic assays (e.g., viscoelastic testing, turbidity-based plasma clot lysis), and fluorogenic plasmin generation (PG). However, evidence on the relationship between hyperfibrinolytic profiles and bleeding severity is inconsistent, and, although found in some coagulation factor deficiencies, has not been universally observed. In BDUC, increased tPA activity and paradoxical increases in thrombin-activatable fibrinolysis inhibitor and α2-AP have been reported. Some studies reported no change in PAI-1 levels, while others observed reduced PAI-1 levels in a significant subset of patients. The tPA-ROTEM (tPA-rotational thromboelastometry) assay identified a hyperfibrinolytic profile in up to 20% of BDUC patients. PG analysis revealed a paradoxically reduced peak plasmin, but showed strong predictive power in differentiating BDUC patients from healthy controls. Although global fibrinolytic assays may help identify hyperfibrinolytic profiles as a potential cause of increased bleeding in some MBD or BDUC patients, the utility of measuring fibrinolytic factors requires further investigation. Tranexamic acid is commonly used to treat hereditary hyperfibrinolysis and is also recommended in MBD/BDUC patients prior to hemostatic challenges.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144013592","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
Pathophysiology and Management of Cerebral Venous Thrombosis. 脑静脉血栓形成的病理生理及治疗。
IF 2.7 4区 医学
Hamostaseologie Pub Date : 2025-04-08 DOI: 10.1055/a-2518-9103
Mariana B Morais, Diana Aguiar de Sousa
{"title":"Pathophysiology and Management of Cerebral Venous Thrombosis.","authors":"Mariana B Morais, Diana Aguiar de Sousa","doi":"10.1055/a-2518-9103","DOIUrl":"https://doi.org/10.1055/a-2518-9103","url":null,"abstract":"<p><p>Cerebral venous thrombosis (CVT) is a less common type of stroke that can occur across all age groups but predominantly affects children and young adults. Diagnosis is often challenging due to the nonspecific and variable clinical presentation. The disease course is heterogeneous, with CVT-related parenchymal lesions developing in approximately 50 to 60% of cases. Despite some advancements, significant gaps persist in understanding the pathophysiology of CVT, including the mechanisms underlying brain injury. Anticoagulation is the cornerstone of CVT treatment, but strategies targeting secondary mechanisms of parenchymal damage are still lacking. Here, the current state of the field is briefly reviewed, with the aim to introduce a wide audience (neuroscientists and clinicians alike) to the disease and inform clinical practice and future research.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143813039","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
Dabigatran hemmt die Bindung von Thrombin an Blutplättchen - ein neuer Wirkmechanismus. 达比加坦抑制凝血酶与血小板的结合，这是一种新的作用机制。
IF 2.7 4区 医学
Hamostaseologie Pub Date : 2025-04-01 Epub Date: 2025-05-07 DOI: 10.1055/s-0045-1809294
{"title":"Dabigatran hemmt die Bindung von Thrombin an Blutplättchen - ein neuer Wirkmechanismus.","authors":"","doi":"10.1055/s-0045-1809294","DOIUrl":"https://doi.org/10.1055/s-0045-1809294","url":null,"abstract":"","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"45 2","pages":"125"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144053150","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
Disorders of Thrombosis and Hemostasis in Cancer. 癌症中血栓和止血障碍。
IF 2.7 4区 医学
Hamostaseologie Pub Date : 2025-04-01 Epub Date: 2025-05-07 DOI: 10.1055/a-2447-6537
Christina Hart, Minna Voigtlaender
{"title":"Disorders of Thrombosis and Hemostasis in Cancer.","authors":"Christina Hart, Minna Voigtlaender","doi":"10.1055/a-2447-6537","DOIUrl":"https://doi.org/10.1055/a-2447-6537","url":null,"abstract":"<p><p>Cancer-associated thrombosis (CAT) remains a critical concern in hematology and oncology, contributing significantly to morbidity and mortality. The interplay between malignancy and hemostasis has been extensively studied, yet it continues to present clinical challenges and opportunities for advancement in prevention, treatment, and management. This special issue of <i>Haemostaseologie-Progress in Haemostasis-Disorders of Thrombosis and Hemostasis in Cancer</i> brings together seven review articles that explore diverse aspects of CAT, shedding light on current practices and emerging trends.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":"45 2","pages":"122-124"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143997004","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
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