{"title":"Annoucements","authors":"","doi":"10.1016/S1538-7836(24)00683-4","DOIUrl":"10.1016/S1538-7836(24)00683-4","url":null,"abstract":"","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"22 12","pages":"Page 3652"},"PeriodicalIF":5.5,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142748176","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Intron 22 inversions: cross-reactive material or no cross-reactive material?","authors":"Bhavya S. Doshi","doi":"10.1016/j.jtha.2024.08.008","DOIUrl":"10.1016/j.jtha.2024.08.008","url":null,"abstract":"","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"22 12","pages":"Pages 3400-3402"},"PeriodicalIF":5.5,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142748177","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Reply to the comments on “Four years into the pandemic, managing COVID-19 patients with acute coagulopathy: what have we learned?” from Wada et al.","authors":"Toshiaki Iba , Jerrold H. Levy","doi":"10.1016/j.jtha.2024.09.010","DOIUrl":"10.1016/j.jtha.2024.09.010","url":null,"abstract":"","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"22 12","pages":"Pages 3645-3646"},"PeriodicalIF":5.5,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142748500","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Daniel Steiner , Stephan Nopp , Timothy Hoberstorfer , Ingrid Pabinger , Benedikt Weber , Cihan Ay
{"title":"Anxiety in patients with venous thromboembolism: quantification and risk factors in a prospective cohort study","authors":"Daniel Steiner , Stephan Nopp , Timothy Hoberstorfer , Ingrid Pabinger , Benedikt Weber , Cihan Ay","doi":"10.1016/j.jtha.2024.07.016","DOIUrl":"10.1016/j.jtha.2024.07.016","url":null,"abstract":"<div><h3>Background</h3><div>Patients with venous thromboembolism (VTE) are at risk of psychological consequences. However, as opposed to physical sequelae of VTE, mental health issues are understudied.</div></div><div><h3>Objectives</h3><div>To assess anxiety after VTE and investigate associated clinical characteristics.</div></div><div><h3>Methods</h3><div>We conducted a prospective cohort study, including patients with acute deep vein thrombosis (DVT) and/or pulmonary embolism. Patients with cancer, pregnancy, or puerperium were excluded. Anxiety was assessed with the Patient-Reported Outcome Measurement Information System short form 8a. Standardized T-scores were calculated (reference, 50; SD, 10), with higher values indicating more anxiety. We associated clinical characteristics at baseline with T-scores at 3-month follow-up in a multivariable linear regression model. Patient clusters depending on anxiety trajectories were explored.</div></div><div><h3>Results</h3><div>We included 257 patients (38.5% women) with a median (IQR) age of 54.1 (42.2-63.5) years. While mean (SD) T-scores decreased from baseline to follow-up (51.03 [9.18] to 46.74 [8.89]; <em>P</em> < .001), we observed an increase in 23.7% of all patients. Female sex (T-score change, 3.09; 95% CI, 0.96-5.22), older age until 45 years, and anxiety at baseline were associated with increased T-scores at follow-up. VTE history (−1.55; 95% CI, −3.62 to 0.52) and pulmonary embolism (−1.23; 95% CI, −3.16 to 0.69) were associated with reduced T-scores, albeit not reaching statistical significance. In a cluster of older female patients with DVT, anxiety tended to increase over time.</div></div><div><h3>Conclusion</h3><div>While most patients with VTE reported reduced anxiety over time, some patients experienced worsening. Female sex, older age, more anxiety at baseline, no VTE history, and DVT were associated with increased anxiety 3 months after VTE.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"22 12","pages":"Pages 3490-3499"},"PeriodicalIF":5.5,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141913086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Development of an assay using a modified coagulation factor V to measure protein S activity","authors":"Keiko Maruyama, Koichi Kokame","doi":"10.1016/j.jtha.2024.08.010","DOIUrl":"10.1016/j.jtha.2024.08.010","url":null,"abstract":"<div><h3>Background</h3><div>Protein S (PS) is an anticoagulant that functions as a cofactor for activated protein C and the tissue factor pathway inhibitor. PS deficiency is a risk factor for venous thromboembolism. PS activity is commonly measured using clot-based assays involving fibrin and thrombin production, but improvements are needed.</div></div><div><h3>Objectives</h3><div>To develop a new assay for measuring plasma PS activity by quantifying the amount of activated coagulation factor (F)V cleaved by activated protein C.</div></div><div><h3>Methods</h3><div>We designed a recombinant, modified FV (FVm) that mimicked FVa. We analyzed 160 purposively selected plasma samples from the Biobank of the National Cerebral and Cardiovascular Center.</div></div><div><h3>Results</h3><div>The assay using mixed normal and PS-deficient plasma detected FVm cleavage in a PS concentration–dependent manner. The correlation between PS activity, measured using the FVm cleavage assay, and free PS antigen levels was relatively weak. We then sequenced all exons of <em>PROS1</em> from 47 subjects with <60% activity in either the FVm cleavage assay or the clot-based assay. Nonsynonymous variants were identified in 12 of 24 subjects with <60% activity in both assays and in 2 of 7 subjects with <60% activity in the FVm cleavage assay alone. No variants were identified in 16 subjects with <60% activity in the clot-based assay alone. Unlike the clot-based assay, the FVm cleavage assay was not affected by the presence of rivaroxaban in the plasma.</div></div><div><h3>Conclusion</h3><div>An assay using the FVm substrate may be less susceptible to interference and provide a more accurate evaluation of plasma PS activity than clot-based assays.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"22 12","pages":"Pages 3510-3520"},"PeriodicalIF":5.5,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142145868","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Calvin B. van Kwawegen , Ferdows Atiq , Dara Endenburg , Karin Fijnvandraat , Karin P.M. van Galen , Marjon H. Cnossen , Saskia E.M. Schols , Marieke J.H.A. Kruip , Waander L. van Heerde , Joke de Meris , Johanna G. van der Bom , Jeroen Eikenboom , Karina Meijer , Frank W.G. Leebeek
{"title":"Genetic variants, thrombocytopenia, and clinical phenotype of type 2B von Willebrand disease: a median 16-year follow-up study","authors":"Calvin B. van Kwawegen , Ferdows Atiq , Dara Endenburg , Karin Fijnvandraat , Karin P.M. van Galen , Marjon H. Cnossen , Saskia E.M. Schols , Marieke J.H.A. Kruip , Waander L. van Heerde , Joke de Meris , Johanna G. van der Bom , Jeroen Eikenboom , Karina Meijer , Frank W.G. Leebeek","doi":"10.1016/j.jtha.2024.08.028","DOIUrl":"10.1016/j.jtha.2024.08.028","url":null,"abstract":"<div><h3>Background</h3><div>Type 2B von Willebrand disease (VWD) is a bleeding disorder caused by gain-of-function variants in the <em>VWF</em> gene. The laboratory and clinical phenotype of type 2B VWD is heterogeneous.</div></div><div><h3>Objectives</h3><div>We investigated associations between genotype and phenotype over a median of 16 years follow-up in a large cohort of well-characterized patients.</div></div><div><h3>Methods</h3><div>We included 64 genetically confirmed type 2B VWD patients from the national multicenter “Willebrand in the Netherlands” study and retrospectively collected clinical and laboratory data from electronic patient records. We analyzed associations between genotype and thrombocytopenia, bleeding phenotype, and events leading to endothelial activation and von Willebrand factor (VWF) secretion, including surgery, desmopressin administration, pregnancy, and delivery.</div></div><div><h3>Results</h3><div>Thrombocytopenia manifested in 67.2% of patients, with varying occurrences between genetic variants (p.Arg1306Trp: 75.0%, p.Arg1308Cys: 58.3%). The most important determinant of thrombocytopenia was the p.Arg1306Trp <em>VWF</em> variant (odds ratio, 25.1). Platelet counts strongly varied over time and were continuously <150 × 10<sup>9</sup>/L in 37.5% of patients with p.Arg1306Trp vs 8.3% in p.Arg1308Cys. In our analysis, endothelial activation was not an independent determinant (odds ratio, 1.3) for thrombocytopenia occurrence. No association was found between thrombocytopenia and cumulative bleeding scores or annual bleeding rates. Four women showed declining platelet counts in all full-term pregnancies (<em>n</em> = 8) during the third trimester with a sharp decrease in the week before delivery. Postpartum hemorrhage, defined as >500 mL estimated blood loss at delivery, occurred in 5 of 8 deliveries, despite prophylactic treatment with VWF concentrates.</div></div><div><h3>Conclusion</h3><div>This study reveals a strong association between <em>VWF</em> variant p.Arg1306Trp and thrombocytopenia in type 2B VWD patients.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"22 12","pages":"Pages 3460-3472"},"PeriodicalIF":5.5,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142349116","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The evolving landscape of von Willebrand disease diagnosis","authors":"Brooke Sadler","doi":"10.1016/j.jtha.2024.09.028","DOIUrl":"10.1016/j.jtha.2024.09.028","url":null,"abstract":"","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"22 12","pages":"Pages 3389-3391"},"PeriodicalIF":5.5,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142748175","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pavlina Chrysafi , Kevin Barnum , Genevieve M. Gerhard , Thita Chiasakul , Arshit Narang , Megan Mcnichol , Nicoletta Riva , Georg Semmler , Bernhard Scheiner , Stefan Acosta , Pierre-Emmanuel Rautou , Mandy N. Lauw , Jonathan Berry , Walter Ageno , Jeffrey I. Zwicker , Rushad Patell
{"title":"Anticoagulation for splanchnic vein thrombosis in myeloproliferative neoplasms: a systematic review and meta-analysis","authors":"Pavlina Chrysafi , Kevin Barnum , Genevieve M. Gerhard , Thita Chiasakul , Arshit Narang , Megan Mcnichol , Nicoletta Riva , Georg Semmler , Bernhard Scheiner , Stefan Acosta , Pierre-Emmanuel Rautou , Mandy N. Lauw , Jonathan Berry , Walter Ageno , Jeffrey I. Zwicker , Rushad Patell","doi":"10.1016/j.jtha.2024.06.029","DOIUrl":"10.1016/j.jtha.2024.06.029","url":null,"abstract":"<div><h3>Background</h3><div>Optimal anticoagulation management in patients with myeloproliferative neoplasms (MPN) experiencing splanchnic vein thrombosis (SpVT) requires balancing risks of bleeding and recurrent thrombosis.</div></div><div><h3>Objectives</h3><div>We conducted a systematic review and meta-analysis to assess the incidence of bleeding and thrombosis recurrence in patients with MPN-SpVT.</div></div><div><h3>Methods</h3><div>We included retrospective or prospective studies in English with ≥10 adult patients with MPN-SpVT. Outcomes included recurrent venous thrombosis (SpVT and non-SpVT), arterial thrombosis, and major bleeding. Pooled rates per 100 patient years with 95% CIs were calculated by DerSimonian–Laird method using random-effects model.</div></div><div><h3>Results</h3><div>Out of 4624 studies screened, 9 studies with a total of 443 patients were included in the meta-analysis with median follow-up of 3.5 years. In the 364 patients with MPN-SpVT treated with anticoagulation, pooled event rate for major bleeding was 2.8 (95% CI, 1.5-5.1; I<sup>2</sup> = 95%), for recurrent venous thrombosis was 1.4 (95% CI, 0.8–2.2; I<sup>2</sup> = 72%), and for arterial thrombosis was 1.4 (95% CI, 0.6-3.3; I<sup>2</sup> = 92%) per 100 patient years. Among 79 patients (<em>n</em> = 4 studies) who did not receive anticoagulation, pooled event rate for major bleeding was 3.2 (95% CI, 0.7-12.7; I<sup>2</sup> = 97%), for recurrent venous thrombosis 3.5 (95% CI, 1.8-6.4; I<sup>2</sup> = 88%), and for arterial thrombosis rate 1.6 (95% CI, 0.4-6.6; I<sup>2</sup> = 95%) per 100 patient years.</div></div><div><h3>Conclusion</h3><div>Patients with MPN-SpVT treated with anticoagulation have significant risks for both major bleeding and thrombosis recurrence. Further studies are necessary to determine the optimal anticoagulation approach in patients with MPN-SpVT.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"22 12","pages":"Pages 3479-3489"},"PeriodicalIF":5.5,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141913085","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Platelet HMGB1 steers intravascular immunity and thrombosis","authors":"Norma Maugeri , Angelo A. Manfredi","doi":"10.1016/j.jtha.2024.07.030","DOIUrl":"10.1016/j.jtha.2024.07.030","url":null,"abstract":"<div><div>Platelets navigate the fine balance between homeostasis and injury. They regulate vascular homeostasis and drive repair after injury amidst leukocyte extravasation. Crucially, platelets initiate extracellular traps generation and promote immunothrombosis. In chronic human diseases, platelet action often extends beyond its normative role, sparking sustained reciprocal activation of leukocytes and mural cells, culminating in adverse vascular remodeling. Studies in the last decade have spotlighted a novel key player in platelet activation, the high mobility group box 1 (HMGB1) protein. Despite its initial characterization as a chromatin molecule, anucleated platelets express abundant HMGB1, which has emerged as a linchpin in thromboinflammatory risks and microvascular remodeling. We propose that a comprehensive assessment of platelet HMGB1, spanning quantification of content, membrane localization, and accumulation of HMGB1-expressing vesicles in biological fluids should be integral to dissecting and quantifying platelet activation. This review provides evidence supporting this claim and underscores the significance of platelet HMGB1 as a biomarker in conditions associated with heightened thrombotic risks and systemic microvascular involvement, spanning cardiovascular, autoimmune, and infectious diseases.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"22 12","pages":"Pages 3336-3345"},"PeriodicalIF":5.5,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142036172","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Vincenzo La Mura , Massimo Colombo , Graham R. Foster , Paolo Angeli , Wolfgang Miesbach , Robert Klamroth , Glenn F. Pierce , Brian O’Mahony , Ming Y. Lim , Virginia Hernandez-Gea , Michael Makris , Flora Peyvandi
{"title":"The management of liver disease in people with congenital bleeding disorders: guidance from European Association for Haemophilia and Allied Disorders, European Haemophilia Consortium, ISTH, and World Federation of Hemophilia","authors":"Vincenzo La Mura , Massimo Colombo , Graham R. Foster , Paolo Angeli , Wolfgang Miesbach , Robert Klamroth , Glenn F. Pierce , Brian O’Mahony , Ming Y. Lim , Virginia Hernandez-Gea , Michael Makris , Flora Peyvandi","doi":"10.1016/j.jtha.2024.08.018","DOIUrl":"10.1016/j.jtha.2024.08.018","url":null,"abstract":"<div><div>People with bleeding disorders (PWBD) have been exposed to the risk of developing chronic viral hepatitis and cirrhosis after replacement therapy. Today, the advent of new pharmacologic strategies for the control of hemostasis and the efficacious antiviral therapies against hepatitis C virus and hepatitis B virus have significantly reduced this risk. However, the definitive success for liver health in this clinical setting is also influenced by other factors, such as the severity of liver disease at the time of hepatitis B virus/hepatitis C virus antiviral therapy and the exposure to highly prevalent factors of chronic liver damage (eg, metabolic dysfunction and/or alcohol) that can cause a residual risk of complications such as hepatocellular carcinoma, portal hypertension, and liver insufficiency. With this background, a group of experts selected among hepatologists, hematologists, PWBD treaters, and patient representatives produced this practical multisociety guidance for the protection of liver health and the prevention and management of liver complications in PWBD based on the most updated protocols of care.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"22 12","pages":"Pages 3629-3639"},"PeriodicalIF":5.5,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142268552","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}