{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T12:15:23Z","timestamp":1789820123637,"version":"4.0.1"},"reference-count":77,"publisher":"Georg Thieme Verlag KG","issue":"04","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hamostaseologie"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats: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 \u03b12-antiplasmin (\u03b12-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 \u03b12-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.<\/jats:p>","DOI":"10.1055\/a-2561-6786","type":"journal-article","created":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T18:47:52Z","timestamp":1745779672000},"page":"312-319","source":"Crossref","is-referenced-by-count":2,"title":["Accelerated Fibrinolysis: A Tendency to Bleed?"],"prefix":"10.1055","volume":"45","author":[{"given":"Dino","family":"Mehic","sequence":"additional","affiliation":[{"name":"Division of Haematology and Haemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingrid","family":"Pabinger","sequence":"additional","affiliation":[{"name":"Division of Haematology and Haemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johanna","family":"Gebhart","sequence":"additional","affiliation":[{"name":"Division of Haematology and Haemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2025,4,27]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1182\/asheducation.V2012.1.466.3798741","article-title":"Is my patient a bleeder? A diagnostic framework for mild bleeding disorders","volume":"2012","author":"T Quiroga","year":"2012","journal-title":"Hematology (Am Soc Hematol Educ Program)"},{"issue":"04","key":"ref2","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1055\/a-2263-5706","article-title":"Bleeding disorder of unknown cause: a diagnosis of exclusion","volume":"44","author":"D Mehic","year":"2024","journal-title":"Hamostaseologie"},{"issue":"08","key":"ref3","doi-asserted-by":"crossref","first-page":"102242","DOI":"10.1016\/j.rpth.2023.102242","article-title":"Investigating patients for bleeding disorders when most of the \u201cusual\u201d ones have been ruled out","volume":"7","author":"D Mehic","year":"2023","journal-title":"Res Pract Thromb Haemost"},{"issue":"03","key":"ref4","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1111\/hae.13422","article-title":"High proportion of patients with bleeding of unknown cause in persons with a mild-to-moderate bleeding tendency: results from the Vienna Bleeding Biobank (VIBB)","volume":"24","author":"J Gebhart","year":"2018","journal-title":"Haemophilia"},{"issue":"07","key":"ref5","doi-asserted-by":"crossref","first-page":"2059","DOI":"10.1016\/j.jtha.2024.03.005","article-title":"Standardization of definition and management for bleeding disorder of unknown cause: communication from the SSC of the ISTH","volume":"22","author":"R I Baker","year":"2024","journal-title":"J Thromb Haemost"},{"issue":"06","key":"ref6","doi-asserted-by":"crossref","first-page":"102176","DOI":"10.1016\/j.rpth.2023.102176","article-title":"Health-related quality of life is impaired in bleeding disorders of unknown cause: results from the Vienna Bleeding Biobank","volume":"7","author":"D Mehic","year":"2023","journal-title":"Res Pract Thromb Haemost"},{"issue":"04","key":"ref7","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1016\/j.jtha.2022.11.045","article-title":"Variability in International Society on Thrombosis and Haemostasis-Scientific and Standardization Committee endorsed Bleeding Assessment Tool (ISTH-BAT) score with normal aging in healthy females: contributory factors and clinical significance","volume":"21","author":"D Doherty","year":"2023","journal-title":"J Thromb Haemost"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.ejim.2020.04.023","article-title":"The discriminatory power of bleeding assessment tools in adult patients with a mild to moderate bleeding tendency","volume":"78","author":"J Gebhart","year":"2020","journal-title":"Eur J Intern Med"},{"issue":"02","key":"ref9","doi-asserted-by":"crossref","first-page":"100081","DOI":"10.1016\/j.rpth.2023.100081","article-title":"Fibrinolysis: an illustrated review","volume":"7","author":"R A Risman","year":"2023","journal-title":"Res Pract Thromb Haemost"},{"issue":"05","key":"ref10","doi-asserted-by":"crossref","first-page":"2758","DOI":"10.3390\/ijms22052758","article-title":"Assessing plasmin generation in health and disease","volume":"22","author":"A Miszta","year":"2021","journal-title":"Int J Mol Sci"},{"issue":"07","key":"ref11","doi-asserted-by":"crossref","first-page":"2427","DOI":"10.2741\/3864","article-title":"An update on the role of carboxypeptidase U (TAFIa) in fibrinolysis","volume":"16","author":"E Heylen","year":"2011","journal-title":"Front Biosci (Landmark Ed)"},{"issue":"09","key":"ref12","doi-asserted-by":"crossref","first-page":"2209","DOI":"10.1111\/jth.14990","article-title":"A new pedigree with thrombomodulin-associated coagulopathy in which delayed fibrinolysis is partially attenuated by co-inherited TAFI deficiency","volume":"18","author":"S K Westbury","year":"2020","journal-title":"J Thromb Haemost"},{"issue":"08","key":"ref13","doi-asserted-by":"crossref","first-page":"1498","DOI":"10.1111\/jth.14160","article-title":"Hemorrhagic disorders of fibrinolysis: a clinical review","volume":"16","author":"J L Saes","year":"2018","journal-title":"J Thromb Haemost"},{"issue":"04","key":"ref14","doi-asserted-by":"crossref","first-page":"e12511","DOI":"10.1002\/rth2.12511","article-title":"Fibrinolysis and bleeding of unknown cause","volume":"5","author":"D Mehic","year":"2021","journal-title":"Res Pract Thromb Haemost"},{"issue":"04","key":"ref15","doi-asserted-by":"crossref","first-page":"e286","DOI":"10.1097\/HS9.0000000000000286","article-title":"Fundamentals for a systematic approach to mild and moderate inherited bleeding disorders: an EHA consensus report","volume":"3","author":"F Rodeghiero","year":"2019","journal-title":"HemaSphere"},{"issue":"19","key":"ref16","doi-asserted-by":"crossref","first-page":"1795","DOI":"10.1182\/blood.2020010038","article-title":"How I treat bleeding disorder of unknown cause","volume":"138","author":"R I Baker","year":"2021","journal-title":"Blood"},{"issue":"02","key":"ref17","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1055\/s-0044-1789596","article-title":"Inherited disorders of the fibrinolytic pathway: pathogenic phenotypes and diagnostic considerations of extremely rare disorders","volume":"51","author":"M Al-Ghafry","year":"2025","journal-title":"Semin Thromb Hemost"},{"issue":"13","key":"ref18","doi-asserted-by":"crossref","first-page":"7027","DOI":"10.3390\/ijms22137027","article-title":"Bleeding disorders in primary fibrinolysis","volume":"22","author":"M Franchini","year":"2021","journal-title":"Int J Mol Sci"},{"issue":"04","key":"ref19","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1111\/jth.13957","article-title":"Measuring fibrinolysis: from research to routine diagnostic assays","volume":"16","author":"C Longstaff","year":"2018","journal-title":"J Thromb Haemost"},{"issue":"S 01","key":"ref20","doi-asserted-by":"crossref","first-page":"3965","DOI":"10.1182\/blood-2023-185756","article-title":"Utility of the platelet function analyzer (PFA-100) in patients with bleeding disorder of unknown cause","volume":"142","author":"D Mehic","year":"2023","journal-title":"Blood"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"1334","DOI":"10.1016\/S0140-6736(78)91973-6","article-title":"Alpha2-plasmin-inhibitor deficiency (Miyasato disease)","volume":"2","author":"K Koie","year":"1978","journal-title":"Lancet"},{"issue":"03","key":"ref22","first-page":"328","article-title":"Alpha 2-antiplasmin deficiency. An overlooked cause of hemorrhage","volume":"15","author":"G C Griffin","year":"1993","journal-title":"Am J Pediatr Hematol Oncol"},{"issue":"06","key":"ref23","doi-asserted-by":"crossref","first-page":"1250","DOI":"10.1111\/j.1365-2516.2008.01766.x","article-title":"\u03b12-antiplasmin and its deficiency: fibrinolysis out of balance","volume":"14","author":"S L Carpenter","year":"2008","journal-title":"Haemophilia"},{"issue":"07","key":"ref24","doi-asserted-by":"crossref","first-page":"2274","DOI":"10.1182\/blood.V93.7.2274","article-title":"Alpha2-antiplasmin gene deficiency in mice is associated with enhanced fibrinolytic potential without overt bleeding","volume":"93","author":"H R Lijnen","year":"1999","journal-title":"Blood"},{"issue":"04","key":"ref25","doi-asserted-by":"crossref","first-page":"998","DOI":"10.1111\/hae.15059","article-title":"Limited value of testing for factor XIII and \u03b12-antiplasmin deficiency in patients with a bleeding disorder of unknown cause","volume":"30","author":"S Ari\u00ebns","year":"2024","journal-title":"Haemophilia"},{"issue":"03","key":"ref26","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1007\/s00277-016-2893-6","article-title":"Fibrinolysis in patients with a mild-to-moderate bleeding tendency of unknown cause","volume":"96","author":"J Gebhart","year":"2017","journal-title":"Ann Hematol"},{"issue":"05","key":"ref27","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1111\/j.1365-2516.2004.00914.x","article-title":"Haemostatic management of intraoral bleeding in patients with congenital deficiency of alpha2-plasmin inhibitor or plasminogen activator inhibitor-1","volume":"10","author":"Y Morimoto","year":"2004","journal-title":"Haemophilia"},{"issue":"06","key":"ref28","doi-asserted-by":"crossref","first-page":"1255","DOI":"10.1111\/j.1365-2516.2008.01834.x","article-title":"Plasminogen activator inhibitor type 1 deficiency","volume":"14","author":"R Mehta","year":"2008","journal-title":"Haemophilia"},{"issue":"16","key":"ref29","doi-asserted-by":"crossref","first-page":"1695","DOI":"10.2174\/1389450120666190715102510","article-title":"Recognition of plasminogen activator inhibitor type 1 as the primary regulator of fibrinolysis","volume":"20","author":"T Urano","year":"2019","journal-title":"Curr Drug Targets"},{"issue":"26","key":"ref30","doi-asserted-by":"crossref","first-page":"12446","DOI":"10.1073\/pnas.92.26.12446","article-title":"Ovulation efficiency is reduced in mice that lack plasminogen activator gene function: functional redundancy among physiological plasminogen activators","volume":"92","author":"G Leonardsson","year":"1995","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"03","key":"ref31","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1111\/hae.12313","article-title":"The obstetric, gynaecological and fertility implications of homozygous PAI-1 deficiency: single-centre experience","volume":"20","author":"M Heiman","year":"2014","journal-title":"Haemophilia"},{"issue":"01","key":"ref32","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1182\/blood-2010-02-267740","article-title":"Venous thrombosis risk associated with plasma hypofibrinolysis is explained by elevated plasma levels of TAFI and PAI-1","volume":"116","author":"M E Meltzer","year":"2010","journal-title":"Blood"},{"issue":"01","key":"ref33","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1111\/j.1538-7836.2005.01709.x","article-title":"Evaluation of low PAI-1 activity as a risk factor for hemorrhagic diathesis","volume":"4","author":"A Agren","year":"2006","journal-title":"J Thromb Haemost"},{"key":"ref34","first-page":"331","article-title":"Relationships between plasma insulin triglyceride, body mass index, and plasminogen activator inhibitor 1","volume":"13","author":"I Juhan-Vague","year":"1987","journal-title":"Diabete Metab"},{"issue":"06","key":"ref35","doi-asserted-by":"crossref","first-page":"1682","DOI":"10.1161\/01.ATV.20.6.1682","article-title":"Role of cytokines in the regulation of plasminogen activator inhibitor-1 expression and secretion in newly differentiated subcutaneous human adipocytes","volume":"20","author":"M Birgel","year":"2000","journal-title":"Arterioscler Thromb Vasc Biol"},{"issue":"03","key":"ref36","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1055\/s-0038-1649833","article-title":"The 4G\/5G genetic polymorphism in the promoter of the plasminogen activator inhibitor-1 (PAI-1) gene is associated with differences in plasma PAI-1 activity but not with risk of myocardial infarction in the ECTIM study. Etude CasTemoins de I'nfarctus du Mycocarde","volume":"74","author":"S Ye","year":"1995","journal-title":"Thromb Haemost"},{"issue":"05","key":"ref37","doi-asserted-by":"crossref","first-page":"1841","DOI":"10.1182\/blood-2002-07-2181","article-title":"The 4G5G polymorphism in the gene for PAI-1 and the circadian oscillation of plasma PAI-1","volume":"101","author":"J G van der Bom","year":"2003","journal-title":"Blood"},{"issue":"05","key":"ref38","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1016\/j.transci.2019.08.007","article-title":"Inherited disorders of the fibrinolytic pathway","volume":"58","author":"S Jain","year":"2019","journal-title":"Transfus Apher Sci"},{"issue":"24","key":"ref39","doi-asserted-by":"crossref","first-page":"1729","DOI":"10.1056\/NEJM199212103272406","article-title":"Brief report: complete deficiency of plasminogen-activator inhibitor type 1 due to a frame-shift mutation","volume":"327","author":"W P Fay","year":"1992","journal-title":"N Engl J Med"},{"issue":"02","key":"ref40","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1586\/ehm.11.5","article-title":"Quebec platelet disorder","volume":"4","author":"C PM Hayward","year":"2011","journal-title":"Expert Rev Hematol"},{"issue":"07","key":"ref41","doi-asserted-by":"crossref","first-page":"1543","DOI":"10.1182\/blood-2008-08-175216","article-title":"Quebec platelet disorder is linked to the urokinase plasminogen activator gene (PLAU) and increases expression of the linked allele in megakaryocytes","volume":"113","author":"M Diamandis","year":"2009","journal-title":"Blood"},{"issue":"06","key":"ref42","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1111\/ijlh.13302","article-title":"Thrombin generation abnormalities in Quebec platelet disorder","volume":"42","author":"J G Brunet","year":"2020","journal-title":"Int J Lab Hematol"},{"issue":"02","key":"ref43","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1182\/blood.V61.2.267.267","article-title":"A new life-long hemorrhagic disorder due to excess plasminogen activator","volume":"61","author":"N A Booth","year":"1983","journal-title":"Blood"},{"issue":"02","key":"ref44","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1055\/s-0038-1661171","article-title":"Inherited fibrinolytic disorder due to an enhanced plasminogen activator level","volume":"52","author":"J Aznar","year":"1984","journal-title":"Thromb Haemost"},{"issue":"06","key":"ref45","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1093\/ajcp\/102.6.816","article-title":"Life-long bleeding diathesis: effect of orthotopic liver transplantation","volume":"102","author":"J E Humphries","year":"1994","journal-title":"Am J Clin Pathol"},{"key":"ref46","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1007\/s00508-017-1194-y","article-title":"Tranexamic acid for treatment and prophylaxis of bleeding and hyperfibrinolysis","volume":"129","author":"I Pabinger","year":"2017","journal-title":"Wien Klin Wochenschr"},{"issue":"21","key":"ref47","doi-asserted-by":"crossref","first-page":"5663","DOI":"10.1182\/bloodadvances.2024012855","article-title":"Plasmin generation analysis in patients with bleeding disorder of unknown cause","volume":"8","author":"D Mehic","year":"2024","journal-title":"Blood Adv"},{"issue":"23","key":"ref48","doi-asserted-by":"crossref","first-page":"2082","DOI":"10.1182\/blood.2018891192","article-title":"Diagnostic high-throughput sequencing of 2396 patients with bleeding, thrombotic, and platelet disorders","volume":"134","author":"K Downes","year":"2019","journal-title":"Blood"},{"issue":"06","key":"ref49","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1046\/j.1365-2516.2002.00686.x","article-title":"Paradoxical hyperfibrinolysis is associated with a more intensely haemorrhagic phenotype in severe congenital haemophilia","volume":"8","author":"M Gr\u00fcnewald","year":"2002","journal-title":"Haemophilia"},{"issue":"08","key":"ref50","doi-asserted-by":"crossref","first-page":"1603","DOI":"10.1111\/jth.13342","article-title":"Reduced fibrinolytic resistance in patients with factor XI deficiency. Evidence of a thrombin-independent impairment of the thrombin-activatable fibrinolysis inhibitor pathway","volume":"14","author":"M Colucci","year":"2016","journal-title":"J Thromb Haemost"},{"issue":"10","key":"ref51","doi-asserted-by":"crossref","first-page":"1076","DOI":"10.1182\/bloodadvances.2017015123","article-title":"Abnormal plasma clot formation and fibrinolysis reveal bleeding tendency in patients with partial factor XI deficiency","volume":"2","author":"G N Gidley","year":"2018","journal-title":"Blood Adv"},{"issue":"12","key":"ref52","doi-asserted-by":"crossref","first-page":"3222","DOI":"10.1111\/jth.15106","article-title":"Pharmacodynamic monitoring of factor VIII replacement therapy in hemophilia A: combining thrombin and plasmin generation","volume":"18","author":"L LFG Valke","year":"2020","journal-title":"J Thromb Haemost"},{"issue":"04","key":"ref53","doi-asserted-by":"crossref","first-page":"630","DOI":"10.1111\/j.1365-2516.2012.02759.x","article-title":"Retrospective evaluation of bleeding tendency and simultaneous thrombin and plasmin generation in patients with rare bleeding disorders","volume":"18","author":"M Van Geffen","year":"2012","journal-title":"Haemophilia"},{"issue":"06","key":"ref54","doi-asserted-by":"crossref","first-page":"1073","DOI":"10.1111\/hae.13842","article-title":"Thrombin and plasmin generation in patients with plasminogen or plasminogen activator inhibitor type 1 deficiency","volume":"25","author":"J L Saes","year":"2019","journal-title":"Haemophilia"},{"issue":"04","key":"ref55","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1160\/TH13-04-0345","article-title":"Simultaneous measurement of thrombin and plasmin generation to assess the interplay between coagulation and fibrinolysis","volume":"110","author":"T Matsumoto","year":"2013","journal-title":"Thromb Haemost"},{"key":"ref56","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1016\/j.cca.2008.11.006","article-title":"Procarboxypeptidase U (TAFI) and the Thr325Ile proCPU polymorphism in patients with hereditary mucocutaneous hemorrhages","volume":"401","author":"V Matus","year":"2009","journal-title":"Clin Chim Acta"},{"issue":"09","key":"ref57","doi-asserted-by":"crossref","first-page":"1488","DOI":"10.1111\/jth.12645","article-title":"No increased systemic fibrinolysis in women with heavy menstrual bleeding","volume":"12","author":"S Wiewel-Verschueren","year":"2014","journal-title":"J Thromb Haemost"},{"issue":"02","key":"ref58","doi-asserted-by":"crossref","first-page":"e12681","DOI":"10.1002\/rth2.12681","article-title":"Fibrinolytic assays in bleeding of unknown cause: improvement in diagnostic yield","volume":"6","author":"L LFG Valke","year":"2022","journal-title":"Res Pract Thromb Haemost"},{"issue":"09","key":"ref59","doi-asserted-by":"crossref","first-page":"1478","DOI":"10.1111\/jth.14529","article-title":"Thrombin-generating potential, plasma clot formation, and clot lysis are impaired in patients with bleeding of unknown cause","volume":"17","author":"S Hofer","year":"2019","journal-title":"J Thromb Haemost"},{"issue":"04","key":"ref60","doi-asserted-by":"crossref","first-page":"e0125069","DOI":"10.1371\/journal.pone.0125069","article-title":"Increased plasma clot permeability and susceptibility to lysis are associated with heavy menstrual bleeding of unknown cause: a case-control study","volume":"10","author":"P Szczepaniak","year":"2015","journal-title":"PLoS One"},{"issue":"03","key":"ref61","first-page":"e106","article-title":"Evaluation of thromboelastometry, thrombin generation and plasma clot lysis time in patients with bleeding of unknown cause: a prospective cohort study","volume":"26","author":"C SB Veen","year":"2020","journal-title":"Haemophilia"},{"key":"ref62","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1016\/j.thromres.2018.12.004","article-title":"Assessment and determinants of whole blood and plasma fibrinolysis in patients with mild bleeding symptoms","volume":"174","author":"M JA Vries","year":"2019","journal-title":"Thromb Res"},{"issue":"S 01","key":"ref63","doi-asserted-by":"crossref","first-page":"1252","DOI":"10.1182\/blood-2023-174613","article-title":"Fibrinolysis assessment with Tpa-ROTEM in patients with bleeding disorders of unknown cause (BDUC)","volume":"142","author":"A Monard","year":"2023","journal-title":"Blood"},{"issue":"01","key":"ref64","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1186\/s12959-022-00435-6","article-title":"Laboratory evaluation of a new integrative assay to phenotype plasma fibrinolytic system","volume":"20","author":"M Bareille","year":"2022","journal-title":"Thromb J"},{"issue":"01","key":"ref65","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/j.transci.2018.02.020","article-title":"A new assay for global fibrinolysis capacity (GFC): Investigating a critical system regulating hemostasis and thrombosis and other extravascular functions","volume":"57","author":"J Amiral","year":"2018","journal-title":"Transfus Apher Sci"},{"issue":"05","key":"ref66","doi-asserted-by":"crossref","first-page":"e423","DOI":"10.1111\/hae.13051","article-title":"Efficacy of tranexamic acid for the prevention of bleeding in patients with von Willebrand disease and Glanzmann thrombasthenia: a controlled, before and after trial","volume":"22","author":"A Eghbali","year":"2016","journal-title":"Haemophilia"},{"issue":"04","key":"ref67","doi-asserted-by":"crossref","first-page":"583","DOI":"10.1111\/hae.12146","article-title":"Tranexamic acid without prophylactic factor replacement for prevention of bleeding in hereditary bleeding disorder patients undergoing endoscopy: a pilot study","volume":"19","author":"A Davis","year":"2013","journal-title":"Haemophilia"},{"issue":"05","key":"ref68","doi-asserted-by":"crossref","first-page":"1177","DOI":"10.1111\/hae.15078","article-title":"Review of interventions and effectiveness for heavy menstrual bleeding in women with moderate and severe von Willebrand disease","volume":"30","author":"O Turan","year":"2024","journal-title":"Haemophilia"},{"issue":"03","key":"ref69","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1111\/ijlh.13155","article-title":"Investigation of patients with unclassified bleeding disorder and abnormal thrombin generation for physiological coagulation inhibitors reveals multiple abnormalities and a subset of patients with increased tissue factor pathway inhibitor activity","volume":"42","author":"S MacDonald","year":"2020","journal-title":"Int J Lab Hematol"},{"issue":"02","key":"ref70","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1111\/hae.12811","article-title":"Unclassified bleeding disorders: outcome of haemostatic challenges following tranexamic acid and\/or desmopressin","volume":"22","author":"S Obaji","year":"2016","journal-title":"Haemophilia"},{"issue":"11","key":"ref71","doi-asserted-by":"crossref","first-page":"1409","DOI":"10.1055\/s-0041-1726344","article-title":"Outcome of surgical interventions and deliveries in patients with bleeding of unknown cause: an observational study","volume":"121","author":"C SB Veen","year":"2021","journal-title":"Thromb Haemost"},{"issue":"07","key":"ref72","doi-asserted-by":"crossref","first-page":"1757","DOI":"10.1016\/j.jtha.2023.03.006","article-title":"Risk factors for future bleeding in patients with mild bleeding disorders: longitudinal data from the Vienna Bleeding Biobank","volume":"21","author":"D Mehic","year":"2023","journal-title":"J Thromb Haemost"},{"issue":"11","key":"ref73","doi-asserted-by":"crossref","first-page":"2519","DOI":"10.1111\/jth.15871","article-title":"Outcomes and management of pregnancy in women with bleeding disorder of unknown cause","volume":"20","author":"D Castle","year":"2022","journal-title":"J Thromb Haemost"},{"issue":"04","key":"ref74","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1111\/hae.12635","article-title":"Primary postpartum haemorrhage in women with von Willebrand disease or carriership of haemophilia despite specialised care: a retrospective survey","volume":"21","author":"S CM Stoof","year":"2015","journal-title":"Haemophilia"},{"issue":"04","key":"ref75","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1097\/AOG.0b013e3181f20177","article-title":"Tranexamic acid treatment for heavy menstrual bleeding: a randomized controlled trial","volume":"116","author":"A S Lukes","year":"2010","journal-title":"Obstet Gynecol"},{"issue":"10","key":"ref76","doi-asserted-by":"crossref","first-page":"2900","DOI":"10.1016\/j.jtha.2024.05.027","article-title":"Examining variability in the diagnosis and management of people with bleeding disorders of unknown cause: communication from the ISTH SSC Subcommittee on von Willebrand Factor","volume":"22","author":"C Kelly","year":"2024","journal-title":"J Thromb Haemost"},{"issue":"07","key":"ref77","doi-asserted-by":"crossref","first-page":"102585","DOI":"10.1016\/j.rpth.2024.102585","article-title":"How to treat patients with bleeding disorder of unknown cause?","volume":"8","author":"C Mussert","year":"2024","journal-title":"Res Pract Thromb Haemost"}],"container-title":["H\u00e4mostaseologie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2561-6786.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,14]],"date-time":"2025-08-14T23:35:13Z","timestamp":1755214513000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2561-6786"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,4,27]]},"references-count":77,"journal-issue":{"issue":"04","published-online":{"date-parts":[[2025,8,11]]},"published-print":{"date-parts":[[2025,8]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2561-6786","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0720-9355","2567-5761"],"issn-type":[{"value":"0720-9355","type":"print"},{"value":"2567-5761","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,4,27]]}}}