Research and Practice in Thrombosis and Haemostasis最新文献

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Skeletal muscle oxygenation and fluid content in upper extremity postthrombotic syndrome: a proof-of-concept study 上肢血栓形成后综合征骨骼肌氧合和液体含量:一项概念验证研究
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI: 10.1016/j.rpth.2025.103008
Laura Avila , Scott Thomas , Athena Mancini , Gina Wong , Brenna Wong , Leonardo R. Brandão , Angelika Stavrakoukas , Leigh Ward , Robert F. Bentley
{"title":"Skeletal muscle oxygenation and fluid content in upper extremity postthrombotic syndrome: a proof-of-concept study","authors":"Laura Avila ,&nbsp;Scott Thomas ,&nbsp;Athena Mancini ,&nbsp;Gina Wong ,&nbsp;Brenna Wong ,&nbsp;Leonardo R. Brandão ,&nbsp;Angelika Stavrakoukas ,&nbsp;Leigh Ward ,&nbsp;Robert F. Bentley","doi":"10.1016/j.rpth.2025.103008","DOIUrl":"10.1016/j.rpth.2025.103008","url":null,"abstract":"<div><h3>Background</h3><div>Little is known about the pathophysiology of upper extremity (UE) postthrombotic syndrome (PTS).</div></div><div><h3>Objectives</h3><div>We aimed to investigate the effect of PTS severity on UE muscle oxygen saturation (SmO<sub>2</sub>) and fluid content during exercise and recovery in adolescents and young adults.</div></div><div><h3>Methods</h3><div>Eleven cases with unilateral or bilateral UE-PTS who sustained deep vein thrombosis (DVT) in childhood, and 11 age- and sex-matched controls from the general population were recruited for this case-control study. PTS was measured using CAPTSure (0-100-point score). SmO<sub>2</sub> and segmental extracellular fluid content were measured in both UEs using near-infrared spectroscopy (NIRS) and bioimpedance spectroscopy (BIS), respectively. Incremental and constant load UE exercise tests were completed using an ergometer while monitoring NIRS and BIS parameters in both UEs. NIRS and BIS changes throughout exercise and recovery were analyzed using mixed models to account for nested data.</div></div><div><h3>Results</h3><div>Median age of DVT diagnosis among cases was 5.6 years (quartile [Q]1-Q3, 0.2-16.1 years). At the time of the study, median CAPTSure score in the DVT-affected UE was 39 points (Q1-Q3, 27-55 points); 55% of DVTs were associated with a central venous catheter. Mixed models showed that increasing PTS severity was associated with lower SmO<sub>2</sub> during both incremental and constant load exercise and exercise recovery. We found no evidence of fluid accumulation during exercise.</div></div><div><h3>Conclusion</h3><div>The lower SmO<sub>2</sub> suggests that UE-PTS severity is associated with muscle microvascular dysfunction and impaired blood flow during and following exercise.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 6","pages":"Article 103008"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145019487","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Challenges in evaluating thromboembolic risk in acute leukemia: clinical profiling and limitations of current scoring systems 评估急性白血病血栓栓塞风险的挑战:临床分析和当前评分系统的局限性
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI: 10.1016/j.rpth.2025.103017
Luisa V. Carvalho , José Vanildo R. de Oliveira , Raphael B. Melo , Fernanda R. Mendes , Cynthia Rothschild , Elvira D.R.P. Velloso , Vanderson Rocha , Eduardo M. Rego , Fernanda A. Orsi , Wellington F. Silva
{"title":"Challenges in evaluating thromboembolic risk in acute leukemia: clinical profiling and limitations of current scoring systems","authors":"Luisa V. Carvalho ,&nbsp;José Vanildo R. de Oliveira ,&nbsp;Raphael B. Melo ,&nbsp;Fernanda R. Mendes ,&nbsp;Cynthia Rothschild ,&nbsp;Elvira D.R.P. Velloso ,&nbsp;Vanderson Rocha ,&nbsp;Eduardo M. Rego ,&nbsp;Fernanda A. Orsi ,&nbsp;Wellington F. Silva","doi":"10.1016/j.rpth.2025.103017","DOIUrl":"10.1016/j.rpth.2025.103017","url":null,"abstract":"<div><h3>Background</h3><div>Limited evidence is available on risk factors for thrombotic events in newly diagnosed acute leukemia (AL) patients, and predictive tools in this population need further validation.</div></div><div><h3>Objectives</h3><div>To evaluate the incidence of thrombosis in newly diagnosed AL patients and to validate known predictive scores.</div></div><div><h3>Methods</h3><div>We retrospectively included 421 patients between 2009 and 2022. Data on thrombotic events (venous and arterial) were collected from admission to 60 days of follow-up. The Khorana, International Society on Thrombosis and Haemostasis (ISTH) disseminated intravascular coagulation, and Siriraj Acute Myeloid/Lymphoblastic Leukemia scores were applied to the cohort.</div></div><div><h3>Results</h3><div>The cumulative incidence of thrombotic events within 60 days was 15.8%, being higher in the acute lymphoblastic leukemia subgroup (21%). Seventy-two percent of the events occurred within the first 30 days of diagnosis. Superficial vein thrombosis and catheter-related thrombosis comprised most events (38.2% and 19.1%, respectively), while arterial thrombosis represented 11.8%. Obesity and platelet count &gt; 20 × 10<sup>9</sup>/L were associated with an overall risk of thrombosis in this cohort. In the acute myeloid leukemia subset, thrombotic event occurrence was associated with a higher peripheral blast percentage and elevated C-reactive protein levels. The Khorana, ISTH-disseminated intravascular coagulation, and Siriraj Acute Myeloid/Lymphoblastic Leukemia scores were not associated with thrombotic events in AL patients.</div></div><div><h3>Conclusion</h3><div>The incidence of thrombotic events is not negligible, being higher in the induction phase. Further studies and better tools to improve event prediction in this population, such as biomarker development, are needed.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 6","pages":"Article 103017"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145019506","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Antithrombotic strategies and outcomes in neonates and infants with cardiac shunts: a systematic review and meta-analysis 新生儿和婴儿心脏分流的抗血栓策略和结果:系统回顾和荟萃分析
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI: 10.1016/j.rpth.2025.103161
Amy L. Kiskaddon , Neil A. Goldenberg , Marisol Betensky , Joshua W. Branstetter , Dina Ashour , Pamela Williams , Arabela C. Stock , Michael Silvey , Therese M. Giglia , Nhue L. Do
{"title":"Antithrombotic strategies and outcomes in neonates and infants with cardiac shunts: a systematic review and meta-analysis","authors":"Amy L. Kiskaddon ,&nbsp;Neil A. Goldenberg ,&nbsp;Marisol Betensky ,&nbsp;Joshua W. Branstetter ,&nbsp;Dina Ashour ,&nbsp;Pamela Williams ,&nbsp;Arabela C. Stock ,&nbsp;Michael Silvey ,&nbsp;Therese M. Giglia ,&nbsp;Nhue L. Do","doi":"10.1016/j.rpth.2025.103161","DOIUrl":"10.1016/j.rpth.2025.103161","url":null,"abstract":"<div><h3>Background</h3><div>Cardiac shunt thrombosis in neonates and infants remains a concern for shunt failure and mortality. The optimal strategy for thromboprophylaxis remains unknown.</div></div><div><h3>Objectives</h3><div>This systematic review aims to characterize antithrombotic strategies and outcomes in neonates and infants with a cardiac shunt.</div></div><div><h3>Methods</h3><div>MEDLINE, Embase, and Cochrane CENTRAL were searched from inception through July 2024 for studies reporting shunt thrombosis prevalence among infants who received a cardiac shunt. We estimated the pooled prevalence of shunt thrombosis using random-effects meta-analysis. In the subgroup analysis, we evaluated the effects of shunt type and antithrombotic strategies on shunt thrombosis prevalence.</div></div><div><h3>Results</h3><div>A total of 39 studies (29 retrospective, 10 prospective) were included, totaling 4735 patients. The most common shunt type was the modified Blalock-Taussig (<em>n</em> = 2224, 47%). Mortality related to shunt thrombosis occurred in 102 (26.2%) patients with shunt thrombosis. The most common antithrombotic agents in the acute postoperative setting were unfractionated heparin (UFH; <em>n</em> = 1452, 30.7%) and aspirin (<em>n</em> = 1413, 29.3%). The pooled prevalence of shunt thrombosis was 8.4% (95% CI, 6.5%-10.4%) and varied among antithrombotic agents: aspirin 7.4% (95% CI, 4.0%-11.4%), UFH 3.8% (95% CI, 0%-12.3%), or UFH followed by aspirin 6.3% (95% CI, 3.6%-9.4%).</div></div><div><h3>Conclusions</h3><div>This systematic review of nearly 5000 neonates and infants reveals a high rate of mortality associated with shunt thrombosis. Collaborative prospective studies are warranted to evaluate antithrombotic regimen–outcome relationships and prognostic factors for shunt thrombosis and bleeding outcomes in these children.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 6","pages":"Article 103161"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145118344","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Assessment of emicizumab levels in EDTA plasma EDTA血浆中emicizumab水平的评估
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI: 10.1016/j.rpth.2025.103175
Christian Pfrepper , Annelie Siegemund , Tristan Klöter , Hagen Bönigk , Sirak Petros , Thomas Siegemund
{"title":"Assessment of emicizumab levels in EDTA plasma","authors":"Christian Pfrepper ,&nbsp;Annelie Siegemund ,&nbsp;Tristan Klöter ,&nbsp;Hagen Bönigk ,&nbsp;Sirak Petros ,&nbsp;Thomas Siegemund","doi":"10.1016/j.rpth.2025.103175","DOIUrl":"10.1016/j.rpth.2025.103175","url":null,"abstract":"","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 6","pages":"Article 103175"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145158762","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Contemporary trends in maternal outcomes during delivery hospitalizations among pregnancies complicated by von Willebrand disease—a cross-sectional analysis 妊娠合并血管性血友病住院分娩期间产妇结局的当代趋势——横断面分析
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI: 10.1016/j.rpth.2025.103174
Minhazur R. Sarker , Rachel Wiley , Vishesh Khanna , Alexander M. Friedman , Timothy Wen
{"title":"Contemporary trends in maternal outcomes during delivery hospitalizations among pregnancies complicated by von Willebrand disease—a cross-sectional analysis","authors":"Minhazur R. Sarker ,&nbsp;Rachel Wiley ,&nbsp;Vishesh Khanna ,&nbsp;Alexander M. Friedman ,&nbsp;Timothy Wen","doi":"10.1016/j.rpth.2025.103174","DOIUrl":"10.1016/j.rpth.2025.103174","url":null,"abstract":"<div><h3>Background</h3><div>While guideline-based multidisciplinary care is increasingly emphasized for managing von Willebrand disease (VWD) in pregnancy, most outcomes data are derived from outdated studies.</div></div><div><h3>Objectives</h3><div>This study aimed to evaluate temporal trends in the prevalence of VWD, estimate hemorrhagic complication trends with VWD, and examine associations with adverse pregnancy outcomes with VWD.</div></div><div><h3>Methods</h3><div>We conducted a cross-sectional analysis leveraging data from the National Inpatient Sample from 2000 to 2022 and identified VWD delivery hospitalizations using International Classification of Diseases codes. Outcomes included placental abruption or antepartum hemorrhage, postpartum hemorrhage, transfusion, nontransfusion severe maternal morbidity, and cesarean and operative vaginal delivery. Joinpoint regression was used to analyze trends by estimating the average annual percentage change. Unadjusted and adjusted logistic regression models were used to determine the strength of association between VWD and adverse pregnancy outcomes.</div></div><div><h3>Results</h3><div>Among 87,151,596 delivery hospitalizations, 4.2 per 10,000 had a diagnosis of VWD. VWD prevalence rose from 2.1 to 5.1 per 10,000 deliveries between 2000 and 2022 (average annual percentage change, 6.6%; 95% CI, 5.3%-19.5%). Delivery hospitalizations with VWD were associated with increased rates of antepartum hemorrhage, postpartum hemorrhage, transfusion, nontransfusion severe maternal morbidity, and cesarean delivery. Of these associations, during the study period for deliveries with VWD, rates of antepartum hemorrhage and transfusion decreased significantly, and delivery route showed a decrease in operative vaginal delivery.</div></div><div><h3>Conclusion</h3><div>Declining transfusion and antepartum hemorrhage rates suggest improvements in diagnosis and management of VWD during pregnancy. However, stable rates of postpartum hemorrhage rate highlight continued gaps in care. These contemporary, population-level findings will inform preconception counseling and intrapartum planning for individuals with VWD.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 6","pages":"Article 103174"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145157992","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Deleterious variants cluster in the A3 domain of factor VIII in people with severe hemophilia A and inhibitors 在严重血友病A和抑制剂患者中,有害变异聚集在因子VIII的A3结构域
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI: 10.1016/j.rpth.2025.103006
Luciana Werneck Zuccherato , Renan Pedra Souza , Ricardo Mesquita Camelo , Márcio Antônio Portugal Santana , Maíse Moreira Dias , Letícia Lemos Jardim , Andrea Gonçalves de Oliveira , Claudia Santos Lorenzato , Monica Hermida Cerqueira , Vivian Karla Brognoli Franco , Rosangela de Albuquerque Ribeiro , Leina Yukari Etto , Maria do Rosario Ferraz Roberti , Fábia Michelle Rodrigues de Araújo Callado , Maria Aline Ferreira de Cerqueira , Ieda Solange de Souza Pinto , Andrea Aparecida Garcia , Tania Hissa Anegawa , Daniele Campos Fontes Neves , Daniel Gonçalves Chaves , Suely Meireles Rezende
{"title":"Deleterious variants cluster in the A3 domain of factor VIII in people with severe hemophilia A and inhibitors","authors":"Luciana Werneck Zuccherato ,&nbsp;Renan Pedra Souza ,&nbsp;Ricardo Mesquita Camelo ,&nbsp;Márcio Antônio Portugal Santana ,&nbsp;Maíse Moreira Dias ,&nbsp;Letícia Lemos Jardim ,&nbsp;Andrea Gonçalves de Oliveira ,&nbsp;Claudia Santos Lorenzato ,&nbsp;Monica Hermida Cerqueira ,&nbsp;Vivian Karla Brognoli Franco ,&nbsp;Rosangela de Albuquerque Ribeiro ,&nbsp;Leina Yukari Etto ,&nbsp;Maria do Rosario Ferraz Roberti ,&nbsp;Fábia Michelle Rodrigues de Araújo Callado ,&nbsp;Maria Aline Ferreira de Cerqueira ,&nbsp;Ieda Solange de Souza Pinto ,&nbsp;Andrea Aparecida Garcia ,&nbsp;Tania Hissa Anegawa ,&nbsp;Daniele Campos Fontes Neves ,&nbsp;Daniel Gonçalves Chaves ,&nbsp;Suely Meireles Rezende","doi":"10.1016/j.rpth.2025.103006","DOIUrl":"10.1016/j.rpth.2025.103006","url":null,"abstract":"<div><h3>Background</h3><div>Hemophilia A (HA) is an X-linked disorder due to deleterious variants in the factor VIII (FVIII) gene (<em>F8</em>). Few studies from large cohorts have explored <em>F8</em> genotype in people with HA with origins other than North American and European.</div></div><div><h3>Objectives</h3><div>We aimed to evaluate the spectrum of <em>F8</em> variants and haplotypes and affected FVIII domains and chains in the context of inhibitor development in people with severe hemophilia A.</div></div><div><h3>Methods</h3><div>We performed genotyping of intron 1 and intron 22 inversions (Inv22) and <em>F8</em> sequencing using a customized gene panel and whole exome sequencing.</div></div><div><h3>Results</h3><div>We included 265 people with severe hemophilia A. We identified deleterious variants in 98.1% of them, totaling 97 unique mutations, of which 32 (33.0%) are novel. Inv22, nonsense, small insertion/deletion, large deletion, and missense variants accounted for 48.3%, 14.7%, 11.3%, 9.8%, and 7.5% of the variants identified. Variants clustered in the FVIII A3 domain were more often associated with people with HA with inhibitors (INH+) than those without inhibitors (INH−) (21.0% vs 0.0%; <em>P</em> &lt; .001); variants clustered in the A1 domain were more often associated with INH− than with INH+ individuals (33.3% vs 7.7%<em>; P</em> &lt; .001). Inv22 was associated with a 4.8-fold increased risk of developing inhibitors in previously untreated patients (odds ratio, 4.81; 95% CI, 2.02-12.01). Conversely, missense variants protected against inhibitor development (odds ratio, 0.09; 95% CI, 0.01-0.50).</div></div><div><h3>Conclusion</h3><div>These findings highlight the role of <em>F8</em> genotype and specific FVIII domains in inhibitor development in an admixed population of people with HA. This could help engineer therapeutic approaches targeting FVIII molecules with reduced immunogenicity.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 6","pages":"Article 103006"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145045744","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Methodological framework and unified metrics for thrombus resolution analysis in the mouse inferior vena cava stenosis model 小鼠下腔静脉狭窄模型血栓溶解分析的方法学框架和统一指标
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI: 10.1016/j.rpth.2025.103009
Ivan Budnik , Mariia Kumskova , Daniel Thedens , Anil K. Chauhan
{"title":"Methodological framework and unified metrics for thrombus resolution analysis in the mouse inferior vena cava stenosis model","authors":"Ivan Budnik ,&nbsp;Mariia Kumskova ,&nbsp;Daniel Thedens ,&nbsp;Anil K. Chauhan","doi":"10.1016/j.rpth.2025.103009","DOIUrl":"10.1016/j.rpth.2025.103009","url":null,"abstract":"<div><h3>Background</h3><div>The inferior vena cava (IVC) stenosis model in mice is widely used in venous thrombosis research. However, high variability in thrombus size, reliance on discrete evaluation time points that vary across studies, and the absence of a unified measure of thrombus resolution dynamics hinder the preclinical assessment of candidate treatments designed to accelerate thrombus resolution.</div></div><div><h3>Objectives</h3><div>To develop a methodological framework for assessing thrombus resolution dynamics in the IVC stenosis model.</div></div><div><h3>Methods</h3><div>Wild-type mice, neutrophil-specific integrin α9-deficient mice (α9<sup>fl/fl</sup>Mrp8Cre<sup>+</sup>), and littermate control (α9<sup>fl/fl</sup>) mice were subjected to IVC stenosis. Thrombus resolution was monitored using magnetic resonance angiography on days 2, 7, 14, and 21 postsurgery in the same mouse cohorts. Linear and nonlinear (exponential decay) mixed-effects models were fitted to analyze thrombus resolution.</div></div><div><h3>Results</h3><div>Ligation of the IVC back branches improved the consistency of thrombus formation in wild-type mice by reducing length variance, independently of surgeon variability. The proportional thrombus resolution rate in wild-type mice was 10.5% per day, with a time to half-maximum thrombus volume of 6.3 days (from day 2 postsurgery). Although neutrophil-specific integrin α9-deficient mice exhibited smaller initial thrombus volumes compared with controls, no difference in thrombus resolution dynamics was observed between the 2 mouse cohorts.</div></div><div><h3>Conclusion</h3><div>Integrating the IVC stenosis model enhanced by back-branch ligation with serial imaging and statistical modeling to derive unified thrombus resolution metrics—proportional resolution rate and time to half-maximum thrombus volume—provides a robust methodological framework. This framework may facilitate the design of preclinical trials using these metrics as standardized outcome measures.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 6","pages":"Article 103009"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145045820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Algorithm-based effective recognition of acquired hemophilia A and other causes of isolated prolonged activated partial thromboplastin time within large blood sample series 基于算法的有效识别获得性血友病A和其他原因在大样本系列中分离的活化部分凝血活素时间延长
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI: 10.1016/j.rpth.2025.103163
Paul Turincev , Francisco Gomez , Christine Coutaz , Stéphane Quarroz , Lorenzo Alberio , Nathalie Rufer
{"title":"Algorithm-based effective recognition of acquired hemophilia A and other causes of isolated prolonged activated partial thromboplastin time within large blood sample series","authors":"Paul Turincev ,&nbsp;Francisco Gomez ,&nbsp;Christine Coutaz ,&nbsp;Stéphane Quarroz ,&nbsp;Lorenzo Alberio ,&nbsp;Nathalie Rufer","doi":"10.1016/j.rpth.2025.103163","DOIUrl":"10.1016/j.rpth.2025.103163","url":null,"abstract":"<div><h3>Background</h3><div>Among the various causes of isolated prolonged activated partial thromboplastin time (aPTT), acquired hemophilia A (AHA) is a rare disorder whose timely recognition is often missed clinically. Consequently, there is a need for a laboratory approach that allows for rapid and reliable evaluation of the causes of an isolated prolonged aPTT within large series of blood samples without specific diagnostic hypotheses.</div></div><div><h3>Objectives</h3><div>To create and implement an automated sequential laboratory-based algorithm to facilitate early AHA diagnosis.' in a new line below the Background section in the Abstract.</div></div><div><h3>Methods</h3><div>A multistep automated algorithm was developed and validated to target blood samples sent for routine coagulation assays, actively excluding samples with an underlying anticoagulant treatment, selecting those with an isolated prolonged aPTT, and detecting clinically relevant causes.</div></div><div><h3>Results</h3><div>The main causes of an isolated prolonged aPTT were anticoagulant drugs and factor (F)XII deficiency/consumption (∼75% of cases). Selection of samples with an isolated prolonged aPTT was achieved by the first 5 automated steps (24 h/7 d), based on routinely available data. During a prospective 3.25-year trial, this strategy enabled the identification of 0.5% (1816/359,229) of samples. After FXII testing (step 6) and exclusion of undisclosed anti-FXa activity (step 7), evaluation of specific causes identified 6.5% (119/1816) of FVIII-deficient samples, including known cases of congenital hemophilia or von Willebrand disease, and 3 new cases of AHA. Since its routine implementation, 4 additional patients with AHA were identified. In all 7 patients, the laboratory-based diagnosis was achieved on the first day of hospitalization without any preceding clinical suspicion for AHA.</div></div><div><h3>Conclusion</h3><div>Our sequential laboratory-based algorithm enables the rapid and accurate identification of rare causes of isolated prolonged aPTT, which can lead to clinically significant bleeding disorders, such as acquired hemophilia.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 6","pages":"Article 103163"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145157993","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Beyond the count: platelet dysfunction profile in pediatric trauma patients 计数之外:儿科创伤患者血小板功能障碍概况
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI: 10.1016/j.rpth.2025.103160
Ronit Kar , Erin V. Feeney , Jack R. Killinger , Katrina M. Morgan , Devin M. Dishong , Katelin C. Rahn , Rassam M.G. Rassam , Grace A. Ballentine , Philip C. Spinella , Matthew D. Neal , Barbara A. Gaines , Susan M. Shea , Christine M. Leeper
{"title":"Beyond the count: platelet dysfunction profile in pediatric trauma patients","authors":"Ronit Kar ,&nbsp;Erin V. Feeney ,&nbsp;Jack R. Killinger ,&nbsp;Katrina M. Morgan ,&nbsp;Devin M. Dishong ,&nbsp;Katelin C. Rahn ,&nbsp;Rassam M.G. Rassam ,&nbsp;Grace A. Ballentine ,&nbsp;Philip C. Spinella ,&nbsp;Matthew D. Neal ,&nbsp;Barbara A. Gaines ,&nbsp;Susan M. Shea ,&nbsp;Christine M. Leeper","doi":"10.1016/j.rpth.2025.103160","DOIUrl":"10.1016/j.rpth.2025.103160","url":null,"abstract":"<div><h3>Background</h3><div>Existing clinical coagulation assays are inadequate to evaluate posttraumatic platelet dysfunction in children.</div></div><div><h3>Objectives</h3><div>To elucidate changes in flow-dependent platelet function after injury in children using a microfluidic assay.</div></div><div><h3>Methods</h3><div>We enrolled 18 children who presented with highest-acuity trauma activation and 10 healthy children at an academic pediatric center. For the control cohort, outpatient blood samples were collected, and for the trauma cohort, blood was collected in the trauma bay for conventional coagulation tests, thromboelastography, von Willebrand factor A1 domain activity, and a microfluidic assay using a stenotic channel (shear rate = 3500 s<sup>−1</sup>).</div></div><div><h3>Results</h3><div>The trauma cohort characteristics were 67% male, median (IQR) age 6 years (4-12), median (IQR) injury severity score 17 (9-26), and 78% blunt mechanism. In total, 50% required blood transfusion and 11% (2/18) died. While thromboelastography maximum amplitude (59.9 ± 6.1 mm) and platelet counts (306.3 ± 111.1 × 10<sup>3</sup> cells/μL) were within normal limits, the microfluidic assay revealed significantly lower platelet deposition in the trauma cohort versus the control cohort (1.18 ± 0.25 vs 3.06 ± 0.82 mean fluorescence intensity fold change; <em>P</em> &lt; .0001). Lower levels of platelet deposition correlated with receipt of blood transfusion within 6 hours of arrival (<em>r</em> = −0.44, <em>P</em> = .048). von Willebrand factor A1 domain activity was not different between groups and was not correlated with platelet deposition based on mean fluorescence intensity fold change (<em>R</em><sup>2</sup> &lt; 0.0001).</div></div><div><h3>Conclusion</h3><div>Posttraumatic platelet dysfunction was observed by utilizing a microfluidic assay in a pediatric trauma cohort. Further study is needed to understand the underlying mechanisms and significance of posttraumatic platelet dysfunction in children.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 6","pages":"Article 103160"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145158761","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Turning up the heat: optimizing Bethesda assays for efanesoctocog alfa monitoring 调高温度:优化Bethesda法用于efanesoccog alfa监测
IF 3.4 3区 医学
Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI: 10.1016/j.rpth.2025.102992
Agathe Herb , Jordan Wimmer , Dominique Desprez , Olivier Feugeas , Laurent Mauvieux , Laurent Sattler
{"title":"Turning up the heat: optimizing Bethesda assays for efanesoctocog alfa monitoring","authors":"Agathe Herb ,&nbsp;Jordan Wimmer ,&nbsp;Dominique Desprez ,&nbsp;Olivier Feugeas ,&nbsp;Laurent Mauvieux ,&nbsp;Laurent Sattler","doi":"10.1016/j.rpth.2025.102992","DOIUrl":"10.1016/j.rpth.2025.102992","url":null,"abstract":"","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"9 6","pages":"Article 102992"},"PeriodicalIF":3.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144888878","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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