Research and Practice in Thrombosis and Haemostasis最新文献

{"title":"Maternal thrombocytopenia is not predictive of neonatal thrombocytopenia: a single-center Irish study","authors":"Ligia Nechifor ,&nbsp;Daniel O’Reilly ,&nbsp;John O’Loughlin ,&nbsp;Fionnuala Ní Áinle ,&nbsp;Naomi Mc Callion ,&nbsp;Lyudmyla Zakharchenko","doi":"10.1016/j.rpth.2024.102622","DOIUrl":"10.1016/j.rpth.2024.102622","url":null,"abstract":"<div><h3>Background</h3><div>Maternal thrombocytopenia during pregnancy is common. However, the relationship between maternal and neonatal thrombocytopenia is poorly understood.</div></div><div><h3>Objectives</h3><div>We aimed to determine whether an association exists between platelet counts of neonates born to mothers with moderate-to-severe thrombocytopenia (&lt;100 × 10⁹/L) and neonatal platelet counts.</div></div><div><h3>Methods</h3><div>We identified records from 557 patients with moderate-to-severe thrombocytopenia (maternal platelet count &lt;100 × 10⁹/L) and the 338 associated newborn charts from 2018 to 2022 in a single large maternity center. Pregnant people with a platelet count of &lt;100 × 10⁹/L prior to delivery during present gestation were included. Any thrombocytopenia that occurred outside of pregnancy or in the postpartum period was excluded. A logistic regression was then generated to examine the association between maternal thrombocytopenia and neonatal thrombocytopenia. A receiver operating characteristic (ROC) curve was generated to assess accuracy of (i) lowest maternal platelet count and (ii) trimester of thrombocytopenia onset in predicting neonatal thrombocytopenia.</div></div><div><h3>Results</h3><div>A total of 550 full blood count assessments were taken in neonates of pregnant people with thrombocytopenia. Sixteen neonates with clinically significant thrombocytopenia (platelet count &lt;100 × 10⁹/L) were identified. A binomial logistic regression was fitted that demonstrated limited association between lowest maternal platelet count and trimester of onset of maternal thrombocytopenia and the development of neonatal thrombocytopenia. An ROC curve was generated to determine the accuracy of maternal platelet count at identifying neonatal thrombocytopenia. The coordinates of the best platelet count threshold for this dataset were then derived from the ROC curve and determined that a threshold of 77.5 × 10⁹/L maternal platelets offered the best accuracy.</div></div><div><h3>Conclusion</h3><div>Neonatal full blood count assessment based on maternal platelet counts of &lt;100 × 10⁹/L has a poor diagnostic yield with no statistically significant association in this cohort on logistic regression analysis. A lower threshold of 77.5 × 10⁹/L may be of higher clinical utility and improve laboratory and clinical workflow.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 8","pages":"Article 102622"},"PeriodicalIF":3.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142759315","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}

{"title":"Efficacy, safety, and quality of life 4 years after valoctocogene roxaparvovec gene transfer for severe hemophilia A in the phase 3 GENEr8-1 trial","authors":"Andrew D. Leavitt ,&nbsp;Johnny Mahlangu ,&nbsp;Priyanka Raheja ,&nbsp;Emily Symington ,&nbsp;Doris V. Quon ,&nbsp;Adam Giermasz ,&nbsp;Maria Fernanda López Fernández ,&nbsp;Gili Kenet ,&nbsp;Gillian Lowe ,&nbsp;Nigel S. Key ,&nbsp;Carolyn M. Millar ,&nbsp;Steven W. Pipe ,&nbsp;Bella Madan ,&nbsp;Sheng-Chieh Chou ,&nbsp;Robert Klamroth ,&nbsp;Jane Mason ,&nbsp;Hervé Chambost ,&nbsp;Flora Peyvandi ,&nbsp;Elaine Majerus ,&nbsp;Dominic Pepperell ,&nbsp;Margareth C. Ozelo","doi":"10.1016/j.rpth.2024.102615","DOIUrl":"10.1016/j.rpth.2024.102615","url":null,"abstract":"<div><h3>Background</h3><div>Valoctocogene roxaparvovec, an adeno-associated virus-mediated gene therapy for severe hemophilia A, enables endogenous factor (F)VIII expression and provides bleed protection.</div></div><div><h3>Objectives</h3><div>Determine valoctocogene roxaparvovec durability, efficacy, and safety 4 years after treatment.</div></div><div><h3>Methods</h3><div>In the phase 3 GENEr8-1 trial, 134 adult male persons with severe hemophilia A without inhibitors and previously using FVIII prophylaxis received a 6 × 10¹³ vg/kg infusion of valoctocogene roxaparvovec. Efficacy endpoints included annualized bleed rate, annualized FVIII infusion rate, FVIII activity, and the Haemophilia-Specific Quality of Life Questionnaire for Adults. Adverse events and immunosuppressant use were assessed. Change from baseline was assessed after participants discontinued prophylaxis (scheduled for week 4).</div></div><div><h3>Results</h3><div>Median follow-up was 214.3 weeks; 2 participants discontinued since the previous data cutoff. Declines from baseline in mean treated annualized bleed rate (−82.6%; <em>P</em> &lt; .0001) and annualized FVIII infusion rate (−95.5%; <em>P</em> &lt; .0001) were maintained from previous years in the primary analysis population of 112 participants who enrolled from a noninterventional study. During year 4, 81 of 110 rollover participants experienced 0 treated bleeds. Week 208 mean and median chromogenic FVIII activity were 16.1 IU/dL and 6.7 IU/dL, respectively, in 130 modified intention-to-treat participants. Seven participants resumed prophylaxis since the previous data cutoff. Mean change from baseline to week 208 in Haemophilia-Specific Quality of Life Questionnaire for Adults Total Score (<em>P</em> &lt; .0001) remained clinically meaningful for modified intention-to-treat participants. Alanine aminotransferase elevation was the most common adverse event during year 4 (56/131 participants); none required immunosuppressants.</div></div><div><h3>Conclusion</h3><div>Valoctocogene roxaparvovec provides persistent FVIII expression, hemostatic control, and health-related quality of life improvements with no new safety signals.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 8","pages":"Article 102615"},"PeriodicalIF":3.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142759316","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}

{"title":"The anticoagulant effects of milvexian, a novel small molecule factor XIa inhibitor, are neutralized by activated prothrombin complex concentrates and recombinant factor VIIa in human plasma and whole blood in vitro","authors":"Matthew Bunce,&nbsp;Zheng Huang Devine,&nbsp;Madhu Chintala","doi":"10.1016/j.rpth.2024.102600","DOIUrl":"10.1016/j.rpth.2024.102600","url":null,"abstract":"<div><h3>Background</h3><div>The development of anticoagulants that provide antithrombotic efficacy without a concomitant bleeding risk remains an unmet clinical need in thrombosis. Although direct oral anticoagulants (DOACs) have a reduced incidence of major bleeding compared with warfarin, they still carry a bleeding risk, resulting in a suboptimal therapeutic index. Epidemiologic data suggest that inhibiting activated factor XI (FXIa) may offer an improved safety profile with respect to bleeding risk compared with current-generation DOACs. Additionally, a phase II trial of milvexian in patients undergoing elective total knee replacement demonstrated robust dose-dependent efficacy with no statistically significant increase in bleeding. Nevertheless, the ability to rapidly and effectively correct FXIa inhibitor–induced anticoagulation may still be important in situations where patients experience uncontrolled bleeding or require emergency surgery.</div></div><div><h3>Objectives</h3><div>We assessed the ability to normalize the anticoagulant effects of the novel small-molecule FXIa inhibitor milvexian (BMS-986177/JNJ-70033093) <em>in vitro</em> using commercially available prohemostatic agents.</div></div><div><h3>Methods</h3><div>Milvexian-associated anticoagulation and correction was evaluated in activated partial thromboplastin time clotting assays, thromboelastography, and kaolin-initiated thrombin generation assays.</div></div><div><h3>Results</h3><div>Activated prothrombin complex concentrates (PCCs) and recombinant factor (rF)VIIa corrected the anticoagulant effects of milvexian in activated partial thromboplastin time clotting assays, thromboelastography, and kaolin-initiated thrombin generation assays. In contrast, other agents, including PCCs, rFIX, and rFVIII, demonstrated either modest or no correction of milvexian-associated anticoagulation.</div></div><div><h3>Conclusion</h3><div>This study demonstrated that currently available activated PCCs and rFVIIa normalize the anticoagulation induced by milvexian <em>in vitro</em>. The clinical utility of these agents remains to be established.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 8","pages":"Article 102600"},"PeriodicalIF":3.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142748651","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}

{"title":"Bleeding disorder of unknown cause: an illustrated review on current practice, knowledge gaps, and future perspectives","authors":"Amaury L.L. Monard ,&nbsp;Caroline M.A. Mussert ,&nbsp;Tirsa T. van Duijl ,&nbsp;Marieke J.H.A. Kruip ,&nbsp;Yvonne M.C. Henskens ,&nbsp;Maartje van den Biggelaar ,&nbsp;Roger E.G. Schutgens ,&nbsp;Saskia E.M. Schols ,&nbsp;Karin J. Fijnvandraat ,&nbsp;Karina Meijer ,&nbsp;Paul L. den Exter ,&nbsp;Laurens Nieuwenhuizen ,&nbsp;Iris van Moort ,&nbsp;Ross I. Baker ,&nbsp;James S. O’Donnell ,&nbsp;Marjon H. Cnossen ,&nbsp;Floor C.J.I. Heubel-Moenen ,&nbsp;BDUC-iN Study group","doi":"10.1016/j.rpth.2024.102625","DOIUrl":"10.1016/j.rpth.2024.102625","url":null,"abstract":"<div><div>In more than half of the individuals with a clinically relevant bleeding tendency who are referred to hemostasis experts, no biological etiology can be found after extensive laboratory testing. These persons are diagnosed with an unexplained bleeding tendency or “bleeding disorder of unknown cause” (BDUC). The mucocutaneous bleeding phenotype of individuals with BDUC is generally comparable to that of individuals with inherited bleeding disorders such as von Willebrand disease or platelet function disorders. BDUC definitions applied in literature are heterogeneous, but all comprise 2 main criteria: (1) there is an increased bleeding tendency based on the clinical view of the physician and/or an increased bleeding score; (2) no abnormalities are found with available hemostasis laboratory tests. This is reflected in the recent published BDUC definition by the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis, stating that BDUC is a diagnosis of exclusion, characterized by normal hemostatic investigations despite a clinically significant bleeding tendency. Importantly, other nonhemostatic and acquired causes of bleeding should be excluded, but details on exclusion criteria and associated diagnostic testing remain undefined. Patients and health care providers are challenged by the uncertainty and lack of formal diagnosis particularly as there is no clear consensus regarding treatment. Research on the diagnostic value of new laboratory tests in individuals with BDUC has not yet been productive. In this illustrative review, the current practice and knowledge gaps in BDUC are addressed, previous research on BDUC is outlined and future directions with outstanding questions for future research in BDUC are highlighted.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 8","pages":"Article 102625"},"PeriodicalIF":3.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142748647","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}

{"title":"External validation of the Leiden Thrombosis Recurrence Risk Prediction models (L-TRRiP) for the prediction of recurrence after a first venous thrombosis in the Heart and Vascular Health study","authors":"J. Louise I. Burggraaf-van Delft ,&nbsp;Kerri L. Wiggins ,&nbsp;Nienke van Rein ,&nbsp;Saskia le Cessie ,&nbsp;Nicholas L. Smith ,&nbsp;Suzanne C. Cannegieter","doi":"10.1016/j.rpth.2024.102610","DOIUrl":"10.1016/j.rpth.2024.102610","url":null,"abstract":"<div><h3>Background</h3><div>Long-term outcome after a first venous thromboembolism (VTE) might be optimized by tailoring anticoagulant treatment duration on individual risks of recurrence and major bleeding. The L-TRRiP models (A–D) were previously developed in data from the Dutch Multiple Environment and Genetic Assessment of Risk Factors for Venous thrombosis study to predict VTE recurrence.</div></div><div><h3>Objectives</h3><div>We aimed to externally validate models C and D using data from the United States Heart and Vascular Health (HVH) study.</div></div><div><h3>Methods</h3><div>Data from participants with a first VTE who discontinued initial anticoagulant therapy were used to determine model performance. Missing data were imputed, and results were pooled according to Rubin’s rules. To determine discrimination, Harrell’s C-statistic was calculated. To assess calibration, the observed/expected (O/E) ratio was estimated, and calibration plots were created, in which we accounted for the competing risk of death. A stratified analysis based on age &lt;70 or &gt;70 years was performed.</div></div><div><h3>Results</h3><div>Of 1430 participants from the HVH study, 187 experienced an unprovoked VTE recurrence during follow-up. The C-statistics of L-TRRIP models C and D were 0.62 (95% CI, 0.56-0.67) and 0.61 (95% CI, 0.55-0.67), respectively. The O/E ratio (1.00; 95% CI, 0.84-1.17 and 1.09; 95% CI, 0.91-1.27, respectively) and calibration plots indicated good calibration. The discrimination was similar between participants &lt;70 or &gt;70 years, whereas overall calibration was lower in participants &lt;70 years.</div></div><div><h3>Conclusion</h3><div>The L-TRRiP models showed moderate discrimination and good calibration in a different population and can be used to guide clinical decision making. To assess the added value in daily clinical practice, a management study is needed.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 8","pages":"Article 102610"},"PeriodicalIF":3.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142702287","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}

{"title":"In-depth characterization of N-glycosylation and sialic acid content in fetal and adult fibrinogen","authors":"Tana V. Palomino ,&nbsp;Anastasia Sheridan ,&nbsp;David C. Muddiman ,&nbsp;Ashley C. Brown","doi":"10.1016/j.rpth.2024.102618","DOIUrl":"10.1016/j.rpth.2024.102618","url":null,"abstract":"<div><h3>Background</h3><div>Fetal fibrinogen is a variant present in neonates. Blood products used in neonates are tailored for adults and do not seamlessly integrate into neonatal clots. Increased sialic acid content has been found in fetal fibrinogen compared with adult fibrinogen. However, the extent or location of sialic acids on fibrinogen remains unknown.</div></div><div><h3>Objectives</h3><div>To investigate differences in glycosylation and sialic acid content between fetal and adult fibrinogen.</div></div><div><h3>Methods</h3><div>Glycans were eluted from human cord blood-isolated fetal fibrinogen and commercially available adult fibrinogen using filter-aided <em>N</em>-linked glycan separation. A <span><math><mrow><mi>α</mi></mrow></math></span>, B <span><math><mrow><mi>β</mi></mrow></math></span>, and <span><math><mrow><mi>λ</mi></mrow></math></span> chains were isolated using sodium dodecyl sulfate-polyacrylamide gel electrophoresis, and in-gel enzymatic digestion was performed. Infrared matrix-assisted laser desorption electrospray ionization mass spectrometry was used for analysis.</div></div><div><h3>Results</h3><div>In total, 39 and 22 glycans were detected in fetal and adult fibrinogen, respectively. Fetal fibrinogen glycans were most abundant in the lower molecular weight range &lt;4 kDa. After isolating the Aα, Bβ, and λ chains, increased glycosylation and sialic acid content was found in fetal fibrinogen. Increased glycosylation was detected across all 3 chains, and increased sialic acid content was found in the Bβ chain.</div></div><div><h3>Conclusion</h3><div>Sialylation in the Bβ chain of fetal fibrinogen supports previous findings showing more knob ‘B’ interactions occur in fetal fibrinogen than in adult fibrinogen during clot polymerization. This is also the first detection of glycosylation in the Aα chain of fibrinogen. By elucidating the fibrinogen <em>N</em>-linked glycome, this study found where sialic acid content differs the most between adult and fetal fibrinogen. This can ultimately be used to develop blood products that are neonatal-compatible.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 8","pages":"Article 102618"},"PeriodicalIF":3.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142748652","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}

{"title":"International normalized ratio measurement during perioperative anticoagulation bridging with low-molecular-weight heparin in patients undergoing heart valve replacement surgery","authors":"Liza Rijvers ,&nbsp;Sanna R. Rijpma ,&nbsp;Herbert B. van Wetten ,&nbsp;Yvonne M.C. Henskens ,&nbsp;An K. Stroobants","doi":"10.1016/j.rpth.2024.102616","DOIUrl":"10.1016/j.rpth.2024.102616","url":null,"abstract":"<div><h3>Background</h3><div>Surgical procedures in anticoagulated patients require specific attention due to increased bleeding risk. Preoperative anticoagulation interruption in high-risk patients is often necessary. Bridging anticoagulation with low-molecular-weight heparin (LMWH) minimizes thromboembolic risk, but its effect on international normalized ratio (INR) measurement is not well established, necessitating careful monitoring and individual assessment.</div></div><div><h3>Objectives</h3><div>To investigate the effect of heparin bridging on INR measurements in anticoagulated patients on vitamin K antagonist (VKA) and in <em>in vitro</em> spiking experiments.</div></div><div><h3>Methods</h3><div>Thirty-eight anticoagulated patients on VKA undergoing valve replacement surgery were studied using 2 plasma-based INR assays and 1 whole blood point-of-care INR method at multiple time points after postoperatively resuming VKA. In addition, INR levels in pooled plasma of both normal and VKA-treated individuals were compared, with 7 spiked concentrations of LMWH or unfractionated heparin (UFH) in 4 INR assays.</div></div><div><h3>Results</h3><div>In LMWH-bridged anticoagulated patients, the INR results obtained with HemosIL RecombiPlasTin and point-of-care Coaguchek were significantly higher than those obtained with STA Hepato Prest within 3 days after restart of VKA. After spiking LMWH or UFH in various concentrations into pooled plasma, only the STA Hepato Prest assay showed no interference in INR measurement within the therapeutic range (1.0-2.0 international units/mL) in both VKA and normal plasma. All other assays showed substantial interference, with the Thromborel S assay being the most heparin-sensitive assay.</div></div><div><h3>Conclusion</h3><div>Differences between INR methods are seen within 72 hours after restarting VKA in postoperative patients who receive LMWH bridging. <em>In vitro</em> experiments using LMWH and UFH show the interference of heparin in multiple INR methods, even with concentrations below the suppliers’ stated heparin interference limits.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 8","pages":"Article 102616"},"PeriodicalIF":3.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142757390","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}

{"title":"Prophylaxis in von Willebrand disease with von Willebrand factor concentrate and nonfactor therapies","authors":"Calvin B. van Kwawegen,&nbsp;Frank W.G. Leebeek","doi":"10.1016/j.rpth.2024.102599","DOIUrl":"10.1016/j.rpth.2024.102599","url":null,"abstract":"<div><div>This manuscript summarizes the current status of prophylaxis and novel potential therapies to prevent bleeding in patients with von Willebrand disease (VWD). VWD is the most common inherited bleeding disorder, which is associated mainly with mucocutaneous bleeding and bleeding during surgical and dental interventions. More severely affected VWD patients, mostly those with type 2 and type 3, can also suffer from joint, muscle, and gastrointestinal bleeds. Most patients with mild and moderate VWD are treated with desmopressin. The majority of patients with type 2 and 3 are treated with von Willebrand factor concentrates, with or without factor VIII. These patients suffer from severe and frequent bleeds and may require regular infusions of von Willebrand factor concentrate to prevent bleeding, so-called prophylaxis, 1 to 3 times per week. In this article, we review the current status of prophylaxis in VWD. We will also discuss emerging treatments that may be used as long-term prophylaxis in patients with severe VWD. We include relevant new data on this topic that were presented during the 2024 International Society on Thrombosis and Haemostasis (ISTH) Congress.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 8","pages":"Article 102599"},"PeriodicalIF":3.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142656605","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}

{"title":"Oral invasive procedures in Glanzmann thrombasthenia: a retrospective observational study","authors":"Maxime Delarue ,&nbsp;François Severac ,&nbsp;Martine Soell ,&nbsp;Léa Pierre ,&nbsp;Dominique Desprez ,&nbsp;Bornert Fabien","doi":"10.1016/j.rpth.2024.102619","DOIUrl":"10.1016/j.rpth.2024.102619","url":null,"abstract":"<div><h3>Background</h3><div>Glanzmann thrombasthenia (GT) is a very rare autosomal inherited bleeding disease affecting megakaryocyte lineage with impacts on oral health such as gingival bleeding, which requires specific management protocols. Very few clinical cases have been published in the dental and hematologic literature.</div></div><div><h3>Objectives</h3><div>This study focuses on a series of 21 patients affected specifically by GT and their hemorrhagic prophylaxis management with the use of recombinant activated factor VII (rFVIIa) for dental extractions and full-mouth debridement.</div></div><div><h3>Methods</h3><div>Data were collected from medical and dental records. rFVIIa was administered prophylactically for oral procedures, following a standardized protocol. Each sessions were performed by experienced oral surgeons, and outcomes were analyzed with a focus on bleeding complications and adverse events.</div></div><div><h3>Results</h3><div>Forty-one full-mouth debridements and 176 dental extractions were performed during 102 sessions of dental care in an outpatient setting. A total of 226 injections of rFVIIa was delivered. The mean number of injections was 2.2 (range, 1-4) per dental procedure. The overall rate of bleeding complications was 5.9% (n = 6). All 6 hemorrhagic complications were classified as minor bleeding. No thromboembolic event or allergic reaction was observed.</div></div><div><h3>Conclusion</h3><div>The data presented in this retrospective observational study support the efficacy and safety of rFVIIa for the prevention of bleeding during invasive dental procedures in patients affected by GT. The rFVIIa protocol presented here seems to be a safe and efficient protocol for the prevention of bleeding during invasive oral procedures.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 8","pages":"Article 102619"},"PeriodicalIF":3.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142702284","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}

{"title":"Incidence and risk factors of ischemic stroke in patients with cancer-associated venous thromboembolism: from the Contemporary Management and Outcomes in Patients With Venous Thromboembolism Registry-2","authors":"Toru Sato ,&nbsp;Yoshito Ogihara ,&nbsp;Yugo Yamashita ,&nbsp;Takeshi Morimoto ,&nbsp;Ryuki Chatani ,&nbsp;Kazuhisa Kaneda ,&nbsp;Yuji Nishimoto ,&nbsp;Nobutaka Ikeda ,&nbsp;Yohei Kobayashi ,&nbsp;Satoshi Ikeda ,&nbsp;Kitae Kim ,&nbsp;Moriaki Inoko ,&nbsp;Toru Takase ,&nbsp;Shuhei Tsuji ,&nbsp;Maki Oi ,&nbsp;Takuma Takada ,&nbsp;Kazunori Otsui ,&nbsp;Jiro Sakamoto ,&nbsp;Takeshi Inoue ,&nbsp;Shunsuke Usami ,&nbsp;Kaoru Dohi","doi":"10.1016/j.rpth.2024.102617","DOIUrl":"10.1016/j.rpth.2024.102617","url":null,"abstract":"<div><h3>Background</h3><div>Ischemic stroke is a serious complication in patients with cancer-associated venous thromboembolism (CAVTE), although data remain scarce in the direct oral anticoagulant era.</div></div><div><h3>Objectives</h3><div>This study aimed to investigate the incidence and identify predictive risk factors of ischemic stroke in patients with CAVTE.</div></div><div><h3>Methods</h3><div>From the Contemporary Management and Outcomes in Patients With Venous Thromboembolism Registry-2 enrolling 5197 venous thromboembolism (VTE) patients across 31 centers in Japan between January 2015 and August 2020, we selected 1507 patients with active cancer. We calculated the cumulative incidence function of ischemic stroke accounting for the competing risk of death and investigated risk factors for ischemic stroke in a subdistribution hazard model of multivariable analysis.</div></div><div><h3>Results</h3><div>During a median follow-up period of 1020 days, 71 patients (4.7%) developed ischemic stroke, and the cumulative incidence of ischemic stroke was 4.0% at 1 year and 4.7% at 3 years. Independent risk factors of ischemic stroke included pancreatic cancer (hazard ratio [HR], 4.24; 95% CI, 2.13-8.43), ovarian cancer (HR, 2.82; 95% CI, 1.31-6.08), lung cancer (HR, 2.35; 95% CI, 1.20-4.57), dyslipidemia (HR, 1.76; 95% CI, 1.01-3.09), metastasis (HR, 1.70; 95% CI, 1.02-2.82), higher D-dimer at VTE diagnosis (HR, 1.09; 95% CI, 1.04-1.14), and younger age (HR, 0.84; 95% CI, 0.71-0.999).</div></div><div><h3>Conclusion</h3><div>In this large VTE registry in the direct oral anticoagulant era, the cumulative incidence of ischemic stroke was 4.0% at 1 year and 4.7% at 3 years in patients with CAVTE, and several independent risk factors of ischemic stroke were identified, including pancreatic cancer, ovarian cancer, lung cancer, dyslipidemia, metastasis, higher D-dimer at VTE diagnosis, and younger age.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 8","pages":"Article 102617"},"PeriodicalIF":3.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142702348","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}