Research and Practice in Thrombosis and Haemostasis最新文献

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Hemophilia treatments and the paradox of choice
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI: 10.1016/j.rpth.2025.102726
Mike Makris , Brian O’Mahony
{"title":"Hemophilia treatments and the paradox of choice","authors":"Mike Makris , Brian O’Mahony","doi":"10.1016/j.rpth.2025.102726","DOIUrl":"10.1016/j.rpth.2025.102726","url":null,"abstract":"","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 2","pages":"Article 102726"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143705638","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
2023 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for antiphospholipid syndrome: good for patients or good for papers?
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI: 10.1016/j.rpth.2025.102735
Katrien M.J. Devreese
{"title":"2023 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for antiphospholipid syndrome: good for patients or good for papers?","authors":"Katrien M.J. Devreese","doi":"10.1016/j.rpth.2025.102735","DOIUrl":"10.1016/j.rpth.2025.102735","url":null,"abstract":"<div><div>The American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for antiphospholipid syndrome (APS) is a new set of robust criteria, including clinical and laboratory criteria, to enhance the identification of patients in clinical studies and laboratory research. Based on a scoring system, patients accruing at least 3 points in the clinical and laboratory domains fulfill the classification criteria for APS. They are meant to define homogeneous patient groups for research. They are not to be used in a clinical setting for diagnosis to identify every patient with APS, where it is essential to include those with an atypical clinical presentation and/or antiphospholipid antibodies laboratory test result. These criteria have provoked a debate among workers in the field. Without nuance, the classification criteria cannot be used for diagnosing APS and may be a potential pitfall for clinicians when no difference is made between “classification” and “diagnostic” criteria. Complementing the American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria with the recently available International Society on Thrombosis and Haemostasis guidance on laboratory detection and interpretation of antiphospholipid antibodies for diagnosis of APS has added value. It ensures rigorous research that leads to improvement of patient management and optimal clinical care in routine practice.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 2","pages":"Article 102735"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143777602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Lower early mortality and risk prediction improvement of obesity after acute pulmonary embolism: results from a multicenter cohort analysis with external validation
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI: 10.1016/j.rpth.2025.102718
Romain Chopard , Laurent Bertoletti , Marc Badoz , Nicolas Meneveau , Fiona Ecarnot , Luciano López Jiménez , Olga Madridano , José Antonio Díaz Peromingo , Meritxell López De la Fuente , Manuel Monreal , Gregory Piazza , RIETE Investigators
{"title":"Lower early mortality and risk prediction improvement of obesity after acute pulmonary embolism: results from a multicenter cohort analysis with external validation","authors":"Romain Chopard ,&nbsp;Laurent Bertoletti ,&nbsp;Marc Badoz ,&nbsp;Nicolas Meneveau ,&nbsp;Fiona Ecarnot ,&nbsp;Luciano López Jiménez ,&nbsp;Olga Madridano ,&nbsp;José Antonio Díaz Peromingo ,&nbsp;Meritxell López De la Fuente ,&nbsp;Manuel Monreal ,&nbsp;Gregory Piazza ,&nbsp;RIETE Investigators","doi":"10.1016/j.rpth.2025.102718","DOIUrl":"10.1016/j.rpth.2025.102718","url":null,"abstract":"<div><h3>Background</h3><div>The relationship between obesity (defined as body mass index [BMI] ≥ 30 kg/m<sup>2</sup>) and mortality in venous thromboembolism remains controversial.</div></div><div><h3>Objectives</h3><div>We aimed to compare outcomes after pulmonary embolism (PE) between patients with obesity and nonobese, nonunderweight patients.</div></div><div><h3>Methods</h3><div>Using a multicenter registry of prospectively recorded individual patient data, we compared outcome rates using multivariable logistic or Cox regression for 30-day and 6-month outcomes respectively (etiologic analysis). We assessed the incremental value of adding BMI information on top of the 30-day European Society of Cardiology (ESC) prognostic algorithm (prognostic analysis).</div></div><div><h3>Results</h3><div>We included 2390 patients with BMI of ≥18.5 kg/m<sup>2</sup> (mean age, 66.9 ± 16.8 years; 1188 men [49.7%]); 686 patients [28.7%] were in the obese group. Mortality rates were significantly lower in patients with obesity than that in patients who were nonobese at 30 days (3.2% [95% CI, 2.0-4.8] vs 5.9% [95% CI, 4.8-7.1]), and 6 months (8.1% [95% CI, 6.2-10.4] vs 16.3% [95% CI, 14.6-18.1]). Rates of secondary nonfatal outcomes (including bleeding, recurrent venous thromboembolism, myocardial infarction, and stroke) did not differ between groups. The addition of the obesity information on top of the ESC prognostic model improved global model fit and discriminatory (Harrell C index from 0.636 to 0.657; <em>P</em> = .07) and calibration capacities (<em>P</em> (Hosmer–Lemeshow) = .02 vs .13), yielding significant reclassification (ie, 10.3%) based on the observed mortality rates with the ESC model as reference. Findings were confirmed in an external validation using 35,796 patients with PE from the RIETE registry.</div></div><div><h3>Conclusion</h3><div>We present evidence indicating lower early- and mid-term mortality after PE in patients classified as obese based on BMI, compared with nonobese, nonunderweight patients. BMI should likely be incorporated into algorithms or scoring systems for predicting early mortality following PE.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 2","pages":"Article 102718"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143705389","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Major bleeding and thromboembolism risks of antithrombotic treatment in patients with incident atrial fibrillation/flutter and a history of cancer
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI: 10.1016/j.rpth.2025.102679
Nienke van Rein , Gordon Chu , Menno V. Huisman , Lars Pedersen , Henrik T. Sørensen , Frederikus A. Klok , Suzanne C. Cannegieter
{"title":"Major bleeding and thromboembolism risks of antithrombotic treatment in patients with incident atrial fibrillation/flutter and a history of cancer","authors":"Nienke van Rein ,&nbsp;Gordon Chu ,&nbsp;Menno V. Huisman ,&nbsp;Lars Pedersen ,&nbsp;Henrik T. Sørensen ,&nbsp;Frederikus A. Klok ,&nbsp;Suzanne C. Cannegieter","doi":"10.1016/j.rpth.2025.102679","DOIUrl":"10.1016/j.rpth.2025.102679","url":null,"abstract":"<div><h3>Background</h3><div>Literature shows that atrial fibrillation (AF) patients with a history of cancer have a higher risk of thromboembolism (TE) and major bleeding (MB) compared to patients without. However, cancer type and time between cancer and AF diagnosis is often lacking in such analyses.</div></div><div><h3>Objectives</h3><div>To examine MB and TE rates of AF patients with a prior cancer diagnosis, stratified by cancer type and interval between cancer and AF diagnosis.</div></div><div><h3>Methods</h3><div>This Danish population-based cohort study included all patients aged ≥50 years with incident AF between January 1, 1995, and December 31, 2016, and identified those who had cancer before the AF diagnosis. From hospital and drug prescription databases, data on cancer type, time interval between cancer and AF diagnosis (ie, &lt;1, 1-3, or &gt;3 years), outcomes, and antithrombotic exposure were collected. Follow-up started from the AF diagnosis until the occurrence of an outcome or the end of the 2-year follow-up. Incidence rates (IRs) per 100 patient-years and adjusted hazard ratios (aHRs) with corresponding 95% CIs were calculated using Cox regression.</div></div><div><h3>Results</h3><div>We identified 39,178 patients with incident AF and a prior cancer diagnosis. These patients demonstrated higher MB (IR, 3.35 [3.25-3.45] vs 2.23 [2.29-2.35]) and TE rates (IR, 3.21 [3.11-3.31] vs 2.53 [2.50-2.56]) than those without prior cancer. The higher MB risk in AF patients with a prior cancer diagnosis was observed in all examined time intervals, while a higher TE risk was only observed in those with a cancer diagnosis &lt;1 year prior (aHR, 1.27 [1.16-1.40]). Prior respiratory cancer was associated with increased MB (aHR, 1.37 [1.26-1.48]) and TE risks (aHR, 1.26 [1.15-1.38]).</div></div><div><h3>Conclusion</h3><div>A prior cancer diagnosis confers additional MB and, to a lesser extent and in certain conditions, thromboembolic risks in patients with AF. The type and timing of the prior cancer diagnosis determines the degree of risk.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 2","pages":"Article 102679"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143610930","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Addressing the perimenopause: what’s blood got to do with it?
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI: 10.1016/j.rpth.2025.102698
Briony A. Cutts , Kristy Fennessy
{"title":"Addressing the perimenopause: what’s blood got to do with it?","authors":"Briony A. Cutts ,&nbsp;Kristy Fennessy","doi":"10.1016/j.rpth.2025.102698","DOIUrl":"10.1016/j.rpth.2025.102698","url":null,"abstract":"<div><div>A state of the art lecture titled, “Addressing the Perimenopause: What’s Blood Got to Do with It?” was presented at the International Society on Haemostasis and Thrombosis (ISTH) Congress in 2024. Perimenopause is when fluctuations of previously cyclically regulated hormones occur prior to menopause, resulting in a number of symptoms that can negatively impact a woman’s quality of life. Thrombosis and hemostasis experts are often approached to help investigate and manage clinical issues associated with perimenopause. This includes the safety of using menopause hormonal therapy in a past history or family history of venous thromboembolism, arterial thrombosis or thrombophilia, heavy menstrual bleeding, and iron deficiency anemia. A review of recent literature and clinical practice guidelines was undertaken to help determine the role of iron deficiency anemia in perimenopause, thrombotic risk in the setting of using menopause hormonal therapy, and indications for thrombophilia testing prior to commencing menopause hormonal therapy. Finally, we summarize relevant new data on this topic presented during the ISTH 2024 Congress.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 2","pages":"Article 102698"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143580128","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Persisting thrombomodulin resistance at 3 months after liver transplantation in children with cirrhosis
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI: 10.1016/j.rpth.2025.102709
Marie-Astrid van Dievoet , Clara David , Audrey Dieu , Cedric Hermans , Thierry Pirotte , Jonathan Douxfils , Ton Lisman , Xavier Stephenne
{"title":"Persisting thrombomodulin resistance at 3 months after liver transplantation in children with cirrhosis","authors":"Marie-Astrid van Dievoet ,&nbsp;Clara David ,&nbsp;Audrey Dieu ,&nbsp;Cedric Hermans ,&nbsp;Thierry Pirotte ,&nbsp;Jonathan Douxfils ,&nbsp;Ton Lisman ,&nbsp;Xavier Stephenne","doi":"10.1016/j.rpth.2025.102709","DOIUrl":"10.1016/j.rpth.2025.102709","url":null,"abstract":"<div><h3>Background</h3><div>The coagulation cascade in pediatric cirrhotic patients appears rebalanced, similar to adults, with few true hemostasis-related bleeds or thromboembolic events before liver transplantation. Vascular thrombosis is an important post–liver transplantation complication. Few papers have addressed the recovery of the coagulation cascade after liver transplantation.</div></div><div><h3>Objectives</h3><div>We aimed to assess the coagulation cascade, with both measurement of individual factors and a global hemostasis assay, before living donor liver transplantation and to investigate its recovery 3 months after transplantation, when liver function has normalized.</div></div><div><h3>Methods</h3><div>From January 2022 to July 2023, pediatric cirrhotic patients were prospectively enrolled 1 day before liver transplantation. An age-matched control group was included for comparison. Routine hemostasis tests, levels of coagulation factors and natural anticoagulants, and thrombomodulin-modified thrombin generation were determined on automated coagulation analyzers at inclusion and 3 months after liver transplantation.</div></div><div><h3>Results</h3><div>Twenty-seven pediatric patients with cirrhosis, primarily of cholestatic origin, and 10 controls were enrolled. Sixteen patients were sampled 3 months after liver transplantation. Pediatric end-stage liver disease scores ranged from −10 to 44. A rebalanced coagulation cascade was confirmed in cirrhotic children, indicated by a thrombomodulin-modified thrombin generation assay similar to controls, although with higher interpatient variability. Interestingly, 3 months posttransplant, coagulation was not completely normalized. In the majority of patients resistance to thrombomodulin persisted.</div></div><div><h3>Conclusion</h3><div>This study confirmed a rebalanced coagulation system in pediatric cirrhotic patients before liver transplantation. Three months posttransplant thrombomodulin resistance persisted. Whereas this contributes to thrombotic complications observed after liver transplantation, remains to be elucidated.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 2","pages":"Article 102709"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143739847","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Major bleeding and thromboembolic complications associated with antithrombotic treatment in patients with atrial fibrillation/flutter and incident cancer
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI: 10.1016/j.rpth.2025.102697
Gordon Chu , Nienke van Rein , Menno V Huisman , Lars Pedersen , Henrik T. Sørensen , Suzanne C. Cannegieter , Frederikus A. Klok
{"title":"Major bleeding and thromboembolic complications associated with antithrombotic treatment in patients with atrial fibrillation/flutter and incident cancer","authors":"Gordon Chu ,&nbsp;Nienke van Rein ,&nbsp;Menno V Huisman ,&nbsp;Lars Pedersen ,&nbsp;Henrik T. Sørensen ,&nbsp;Suzanne C. Cannegieter ,&nbsp;Frederikus A. Klok","doi":"10.1016/j.rpth.2025.102697","DOIUrl":"10.1016/j.rpth.2025.102697","url":null,"abstract":"<div><h3>Background</h3><div>Anticoagulant management of patients with atrial fibrillation with active cancer is complex because cancer increases the risk of thrombosis as well as bleeding. Previous studies have investigated the impact of any type of cancer, while outcomes may differ per specific type. We performed the present study to provide more insight into the impact of specific types of cancer on clinical outcomes.</div></div><div><h3>Objectives</h3><div>We examined major bleeding (MB) and thromboembolism (TE) rates associated with antithrombotic treatment in patients with atrial fibrillation/flutter (AF) who develop cancer and examined whether cancer type affected MB and TE risks.</div></div><div><h3>Methods</h3><div>This Danish population-based cohort study included all patients aged ≥ 50 years discharged with incident AF between January 1, 1995, and December 31, 2016, and identified those who subsequently developed cancer. Data on cancer type, outcomes, and antithrombotic exposure were obtained from hospital and drug prescription databases. Follow-up continued from the time of cancer diagnosis until the occurrence of an outcome or the end of the 2-year follow-up. Incidence rates (IRs) per 100 patient-years and adjusted hazard ratios with corresponding 95% CIs were calculated using Cox regression.</div></div><div><h3>Results</h3><div>A total of 22,996 patients with AF with subsequent incident cancer were identified. These patients had higher MB (IR, 5.36 [95% CI, 5.09-5.64] vs 2.27 [95% CI, 2.22-2.32]) and TE (IR, 3.91 [95% CI, 3.68-4.15] vs 2.71 [95% CI, 2.66-2.76]) rates than those without cancer. The higher MB rate was observed across all antithrombotic exposure categories. Urogenital (IR, 6.43 [95% CI, 5.94-6.95]) and intracranial cancer (IR, 6.36 [95% CI, 3.85-9.76]) demonstrated the highest MB rates; hematologic (IR, 4.92 [95% CI, 4.12-5.82]) and gastrointestinal cancer (IR, 4.82 [95% CI, 4.31-5.36]) had the highest TE rates. A particularly high MB rate was observed in patients with AF with gastrointestinal cancer and triple antithrombotic therapy (IR, 39.0 [95% CI, 15.5-79.1]).</div></div><div><h3>Conclusion</h3><div>Patients with AF with certain incident cancer types experienced higher rates of MB and TE than those without cancer. Dual/triple antithrombotic therapy in patients with AF with incident cancer was associated with high bleeding rates, particularly with gastrointestinal cancer.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 2","pages":"Article 102697"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143592077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Real-world bleeding rates on emicizumab: the value of using nationwide digital treatment diary data in clinical research
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI: 10.1016/j.rpth.2025.102717
Martijn R. Brands , Elisabeth M. Taal , Martijn Oude Voshaar , Mariëtte H.E. Driessens , Caroline M.E. van Veen , Marieke J.H.A. Kruip , Paul L. den Exter , Britta A.P. Laros-van Gorkom , Marjet A. Stein-Wit , Kathelijn Fischer , Stephan Meijer , Karina Meijer , Marlène Beijlevelt , Karin Fijnvandraat , Samantha C. Gouw
{"title":"Real-world bleeding rates on emicizumab: the value of using nationwide digital treatment diary data in clinical research","authors":"Martijn R. Brands ,&nbsp;Elisabeth M. Taal ,&nbsp;Martijn Oude Voshaar ,&nbsp;Mariëtte H.E. Driessens ,&nbsp;Caroline M.E. van Veen ,&nbsp;Marieke J.H.A. Kruip ,&nbsp;Paul L. den Exter ,&nbsp;Britta A.P. Laros-van Gorkom ,&nbsp;Marjet A. Stein-Wit ,&nbsp;Kathelijn Fischer ,&nbsp;Stephan Meijer ,&nbsp;Karina Meijer ,&nbsp;Marlène Beijlevelt ,&nbsp;Karin Fijnvandraat ,&nbsp;Samantha C. Gouw","doi":"10.1016/j.rpth.2025.102717","DOIUrl":"10.1016/j.rpth.2025.102717","url":null,"abstract":"<div><h3>Background</h3><div>People with hemophilia in the Netherlands log bleeds and infusions through a digital treatment diary. With the current innovations in hemophilia treatments, the use of patient-reported bleeding data will become increasingly important.</div></div><div><h3>Objective</h3><div>To assess real-world bleeding rates on emicizumab in a nationwide cohort of people with severe hemophilia A, and assess the value of digital treatment diary data.</div></div><div><h3>Methods</h3><div>People with severe hemophilia A of all ages with and without inhibitors using emicizumab who use the digital treatment diary were included. From 2018 to October 2023, data on bleeds treated with clotting factor concentrate were collected from digital treatment diaries and electronic health records. Mean (95% CI) annualized (joint) bleeding rates were calculated using negative-binomial regression analyses. Proportions of people with zero-treated (joint) bleeds were assessed using Kaplan–Meier survival analysis. We calculated the proportion of all bleeds that were recorded in digital treatment diaries.</div></div><div><h3>Results</h3><div>The 232 included persons (median age, 27 years; IQR, 13-51) who used emicizumab for a median of 27 months (IQR, 14-31 months). The mean treated annualized bleeding rate and annualized joint bleeding rate were 1.5 (CI, 1.3-1.8) and 0.8 (CI, 0.6-1.0), respectively. At 24 weeks, 63% had zero-treated bleeds, and 80% had zero-treated joint bleeds. Of treated bleeds, 67% (310/460) were reported in digital treatment diaries.</div></div><div><h3>Conclusion</h3><div>Bleeding rates among Dutch people with severe hemophilia A using emicizumab were comparable to other real-world studies. We formulated recommendations to improve the quality of patient-reported bleeding data, such as establishing guidelines for recording bleeds and improving interoperability.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 2","pages":"Article 102717"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143683445","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A rare case of factor X deficiency induced by valproic acid
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI: 10.1016/j.rpth.2025.102721
Pierre-Antonin Rigon , Vincent Ernest
{"title":"A rare case of factor X deficiency induced by valproic acid","authors":"Pierre-Antonin Rigon ,&nbsp;Vincent Ernest","doi":"10.1016/j.rpth.2025.102721","DOIUrl":"10.1016/j.rpth.2025.102721","url":null,"abstract":"<div><h3>Background</h3><div>Factor X (FX) deficiency (FXD) significantly disrupts coagulation, potentially leading to severe bleeding. While inherited FXD is rare, with a prevalence of 1 in 500,000, acquired FXD is also uncommon and frequently linked to conditions such as light-chain amyloidosis. In rare cases, certain medications can cause FXD.</div></div><div><h3>Key Clinical Question</h3><div>Here, we present a rare case of acquired FXD induced by valproic acid (VPA). This deficiency is associated with the presence of anti-FX antibodies.</div></div><div><h3>Clinical Approach</h3><div>A 65-year-old man undergoing treatment for various conditions, including chronic kidney disease and type 2 diabetes, developed severe FXD (activity &lt;2 U/L) following VPA administration for epilepsy. During FXD, the patient experienced significant bleeding episodes, necessitating FX replacement with prothrombin complex concentrate. Upon discontinuation of VPA, FX activity improved in 9 days, possibly suggesting a role of the drug in FXD. Interestingly, antibodies directed against FX have been identified.</div></div><div><h3>Conclusion</h3><div>This case emphasizes the necessity for clinicians to be vigilant of hemostasis disorders associated with VPA, even though such occurrences are rare.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 2","pages":"Article 102721"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143683447","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Sexuality and bleeding in von Willebrand disease
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI: 10.1016/j.rpth.2025.102712
Calvin B. van Kwawegen , Hester Pastoor , Jeroen Eikenboom , Karin Fijnvandraat , Paula Ypma , Floor C.J.I. Heubel-Moenen , Karin P.M. van Galen , Evelien P. Mauser-Bunschoten , Karina Meijer , Saskia E.M. Schols , Marjon H. Cnossen , Johanna G. van der Bom , Joke de Meris , Ferdows Atiq , Marieke J.H.A. Kruip , Frank W.G. Leebeek
{"title":"Sexuality and bleeding in von Willebrand disease","authors":"Calvin B. van Kwawegen ,&nbsp;Hester Pastoor ,&nbsp;Jeroen Eikenboom ,&nbsp;Karin Fijnvandraat ,&nbsp;Paula Ypma ,&nbsp;Floor C.J.I. Heubel-Moenen ,&nbsp;Karin P.M. van Galen ,&nbsp;Evelien P. Mauser-Bunschoten ,&nbsp;Karina Meijer ,&nbsp;Saskia E.M. Schols ,&nbsp;Marjon H. Cnossen ,&nbsp;Johanna G. van der Bom ,&nbsp;Joke de Meris ,&nbsp;Ferdows Atiq ,&nbsp;Marieke J.H.A. Kruip ,&nbsp;Frank W.G. Leebeek","doi":"10.1016/j.rpth.2025.102712","DOIUrl":"10.1016/j.rpth.2025.102712","url":null,"abstract":"<div><h3>Background</h3><div>Sexuality is a fundamental aspect of quality of life, often impacted by chronic or inherited diseases like von Willebrand disease (VWD), an inherited bleeding disorder characterized by mucosal bleeding, including heavy menstrual bleeding (HMB). To date, no studies have investigated the impact of VWD on sexuality.</div></div><div><h3>Objectives</h3><div>This study aimed to identify sexual restrictions and symptoms in VWD patients, differentiating between men and women and between premenopausal and nonmenstruating women.</div></div><div><h3>Methods</h3><div>We performed a nationwide, multicenter, prospective cohort study, the Willebrand in the Netherlands-Prospective study, including adult VWD patients (&gt;18 years) who completed questionnaires on sexuality and health-related quality of life (SF-36). Additional data were collected via blood tests and a self-reported bleeding assessment tool (International Society on Thrombosis and Haemostasis Bleeding Assessment Tool).</div></div><div><h3>Results</h3><div>We included 549 VWD patients with a median age of 51 years (IQR, 37-66 years), of whom the majority were women (<em>n</em> = 347; 63.2%). Patients were diagnosed with type 1 (57.2%), type 2 (39.2%), or type 3 VWD (3.6%). Sexual restrictions due to VWD were reported by 3.5% of men (<em>n</em> = 7) and 9.8% of women (<em>n</em> = 34; <em>P</em> &lt; .01). Bleeding during sexual activity was reported by 33.1% (<em>n</em> = 115) of women. Premenopausal patients more often reported sexual restrictions than nonmenstruating patients (15.5% vs 5.2%, <em>P</em> = .01), with HMB as the most important determinant (odds ratio, 1.60; 95% CI, 1.12-2.46). Most patients (<em>n</em> = 455; 82.9%) reported that sexuality was not discussed during routine clinic visits.</div></div><div><h3>Conclusion</h3><div>Women with VWD experience more sexual restrictions than men and report more postcoital bleeding than the general population. Premenopausal women are particularly affected, mostly due to HMB. This highlights the need for health care providers to address sexual health during consultations and treat HMB to improve overall care for VWD patients.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 2","pages":"Article 102712"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143705390","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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