Research and Practice in Thrombosis and Haemostasis最新文献

{"title":"Subacute immune-mediated thrombotic thrombocytopenic purpura: prolonged prodromes and delayed diagnosis in 5 cases","authors":"Tara G. Tehrani,&nbsp;David J. Sutton,&nbsp;Richard Whitmill,&nbsp;Muzna Aquil,&nbsp;Amir Shenouda,&nbsp;Aurangzeb Razzak,&nbsp;Humayun Ahmad,&nbsp;Malahat Saeed,&nbsp;Gillian C. Lowe,&nbsp;Phillip L.R. Nicolson","doi":"10.1016/j.rpth.2026.106972","DOIUrl":"10.1016/j.rpth.2026.106972","url":null,"abstract":"<div><h3>Background</h3><div>Immune thrombotic thrombocytopenic purpura (iTTP) is caused by a deficiency in ADAMTS-13 activity and is classically an acute-onset disorder. A subset of patients, however, may experience a more insidious course. Comprehensive reports of these presentations are sparse, and delayed diagnosis has been associated with increased thrombotic complications and mortality.</div></div><div><h3>Objectives</h3><div>To report on 5 cases of subacute iTTP presentations involving a prolonged prodromal period of nonspecific symptoms with gradual decline in platelet counts before acute deterioration and subsequent diagnosis.</div></div><div><h3>Methods</h3><div>This retrospective case series identified delayed iTTP diagnoses in 5 patients with thrombocytopenia (platelets &lt;150 ×10⁹/L) and ADAMTS-13 activity &lt;10 IU/dL managed at a single UK specialist tertiary referral center between 2022 and 2024. Clinical data, laboratory results, treatments, and outcomes were obtained from medical records.</div></div><div><h3>Results</h3><div>The median age was 52 years (range, 3-78), and 3 patients were female. Median time from first nonspecific symptom to diagnosis was 84 days (range, 60-120), compared with 22 days (range, 5-65) from the first documented evidence of microangiopathic hemolytic anemia or thrombotic microangiopathy. All patients were found to have severe thrombocytopenia and ADAMTS-13 activity &lt;10 IU/dL, with 3 showing anti-ADAMTS-13 IgG positivity. All patients were treated with plasma exchange, corticosteroids, rituximab, and caplacizumab; 3 required obinutuzumab for relapsed/refractory low ADAMTS-13 levels.</div></div><div><h3>Conclusion</h3><div>Insidious changes to blood count trends and prodromal symptoms risk being overlooked, delaying treatment and increasing morbidity and mortality. Broader awareness of subacute iTTP presentations outside hematology may improve timely diagnosis and management.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"10 7","pages":"Article 106972"},"PeriodicalIF":3.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149213330","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":"A buffer-positive serotonin release assay pattern is associated with increased mortality in HIT: a retrospective cohort study","authors":"Tarinee Rungjirajittranon,&nbsp;Theodore E. Warkentin,&nbsp;Adam Cuker","doi":"10.1016/j.rpth.2026.106995","DOIUrl":"10.1016/j.rpth.2026.106995","url":null,"abstract":"<div><h3>Background</h3><div>HIT is an anti–platelet factor 4 (PF4) disorder characterized by thrombocytopenia and increased thrombosis risk. The serotonin release assay (SRA) is the gold standard test for diagnosing HIT. The clinical significance of a buffer-positive SRA pattern remains uncertain.</div></div><div><h3>Objectives</h3><div>This study compared characteristics and outcomes between patients with HIT with buffer-negative and buffer-positive SRA patterns.</div></div><div><h3>Methods</h3><div>We conducted a retrospective cohort study at the Hospital of the University of Pennsylvania and affiliated hospitals. Consecutive patients with confirmed HIT, defined as a positive PF4/heparin enzyme-linked immunoassay and platelet-rich plasma-based SRA without a high-heparin inhibition step, were classified by an SRA pattern (buffer-negative vs buffer-positive) and followed for 30 days. The primary outcome was cumulative incidence of thrombosis; secondary outcomes included mortality, major bleeding, and platelet recovery.</div></div><div><h3>Results</h3><div>Of 207 HIT-positive patients, 190 (91.8%) had a buffer-negative and 17 (8.2%) had a buffer-positive SRA pattern. Median anti-PF4/heparin enzyme-linked immunoassay optical density was significantly lower in the buffer-positive group (0.8 vs 2.3; <em>P</em> &lt; .001). Thrombosis incidence was similar between groups (64.7% vs 50.2%; <em>P</em> = .28). The buffer-positive pattern was independently associated with longer time to platelet recovery (9.0 vs 5.0 days; <em>P</em> = .03) and higher 30-day cumulative incidence of death (47.4% vs 17.1%; adjusted hazard ratio, 2.74; <em>P</em> = .03).</div></div><div><h3>Conclusion</h3><div>Among patients with HIT, a buffer-positive SRA pattern was associated with more prolonged thrombocytopenia and increased mortality. Given that our assay used platelet-rich plasma, these findings are hypothesis-generating and require confirmation with the classical washed-platelet SRA.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"10 7","pages":"Article 106995"},"PeriodicalIF":3.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149253980","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":"Evolutionary constraint and population variation across coagulation genes","authors":"Omid Seidizadeh,&nbsp;Himani Chand,&nbsp;Andrea Cairo,&nbsp;Flora Peyvandi","doi":"10.1016/j.rpth.2026.106980","DOIUrl":"10.1016/j.rpth.2026.106980","url":null,"abstract":"<div><h3>Background</h3><div>Hemostasis is a tightly regulated system in which disruption of coagulation genes can cause bleeding or thrombotic disorders. Despite extensive variant discovery, it remains unclear whether coagulation genes share uniform or gene-specific patterns of evolutionary constraint and population variation.</div></div><div><h3>Objectives</h3><div>To characterize evolutionary constraint and population variation across coagulation genes using large-scale genomic data.</div></div><div><h3>Methods</h3><div>We performed a population-genomic analysis of 34 genes associated with inherited bleeding, fibrinolytic, and thrombotic disorders using Genome Aggregation Database (gnomAD) v4.1.1 data from more than 800,000 individuals. We evaluated gene-level constraint metrics, including loss-of-function observed/expected ratios, loss-of-function observed/expected upper bound fraction, missense constraint scores, and synonymous variation metrics. We quantified rare and very rare variant burden after normalization for coding sequence length, assessed predicted functional impact using CADD and REVEL scores, evaluated nucleotide-level evolutionary conservation using phylogenetic <em>P</em> value scores, and characterized ClinVar pathogenic, uncertain, and benign variants identified in gnomAD.</div></div><div><h3>Results</h3><div>Hemostatic genes demonstrated a continuous spectrum of evolutionary constraint rather than uniform intolerance to genetic variation. Genes including <em>F8, F9, F2, VWF, MYH9, SERPINC1,</em> and <em>PROS1</em> showed strong depletion of damaging variation, whereas <em>GP6, GP1BB, F12, VKORC1, MCFD2,</em> and <em>LMAN1</em> showed greater tolerance. Rare variant density differed substantially across genes even after adjustment for coding sequence length. ClinVar pathogenic variants identified in gnomAD were enriched at evolutionarily conserved sites and displayed marked gene-specific differences in variant class distribution, particularly among missense variants.</div></div><div><h3>Conclusions</h3><div>The hemostatic system is shaped by heterogeneous evolutionary pressures, resulting in gene-specific patterns of constraint and variation tolerance. Integrating population genomics, evolutionary conservation, and clinical annotations provides a framework for understanding genetic variation across bleeding, thrombotic, and fibrinolytic disorders.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"10 7","pages":"Article 106980"},"PeriodicalIF":3.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149213333","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":"Platelet-released polyphosphate controls thrombin generation","authors":"Jack M. Kurth,&nbsp;Xu Zhang,&nbsp;Joseph E. Aslan,&nbsp;Owen J.T. McCarty","doi":"10.1016/j.rpth.2026.106953","DOIUrl":"10.1016/j.rpth.2026.106953","url":null,"abstract":"","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"10 7","pages":"Article 106953"},"PeriodicalIF":3.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149213347","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":"Prevalence of qualitative platelet disorders in adolescents and young adults with heavy menstrual bleeding: a single center experience","authors":"Maria Carter-Febres,&nbsp;Cristina Tarango,&nbsp;Ashley Childress,&nbsp;Qin Sun,&nbsp;Jennifer Pomales,&nbsp;Eric S. Mullins","doi":"10.1016/j.rpth.2026.106970","DOIUrl":"10.1016/j.rpth.2026.106970","url":null,"abstract":"<div><h3>Background</h3><div>Heavy menstrual bleeding (HMB) is a chronic condition that impacts an estimated 34% to 37% of the adolescent and young adult (AYA) population. Two of the most common causes of HMB in the AYA population are anovulatory cycles and inherited bleeding disorders (BDOs). Qualitative platelet disorders (QPDs) comprise a substantial portion of these BDOs, though the reported prevalence among AYAs with HMB is variable, with rates ranging from 28% to 49%.</div></div><div><h3>Objectives/Hypothesis</h3><div>Our primary aim was to assess the prevalence of QPDs among AYA individuals with unexplained HMB who were referred to our BDO clinic. In addition, we sought to understand how social determinants of health impacted time to testing for a QPD.</div></div><div><h3>Methods</h3><div>This is a single-site, retrospective chart review of AYAs with unexplained HMB who underwent platelet function testing (PFT), composed of lumiaggregometry, quinacrine uptake/release, CD63 expression, and glycoprotein expression, at our institution. Data points collected included demographics, menstrual history (menarche and menstrual bleeding), which was determined by chart review, laboratory testing (PFT results), and timing from menarche to the first BDO visit, from menarche to PFT, and from first BDO visit to PFT to evaluate the impacts of social determinants of health.</div></div><div><h3>Results</h3><div>A total of 411 individuals were included, and 44 individuals (10%) were ultimately diagnosed with a QPD. Of the 411 individuals, 103 (25%) had abnormal findings on initial PFT, many of which were nonspecific, prompting repeat testing. Repeat testing was performed in 63 of the 411 individuals (15%), and of those, 40 (10%) had normalization of PFT. Although there were no differences in time from menarche to first BDO visit or PFT, Black individuals had a longer median time from first BDO visit to first PFT than White individuals.</div></div><div><h3>Conclusion</h3><div>QPD is less prevalent in individuals with unexplained HMB than previously described. This study highlights the importance of repeating PFT to confirm abnormal results before diagnosing a QPD.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"10 7","pages":"Article 106970"},"PeriodicalIF":3.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149253984","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":"Mouse models to study von Willebrand factor in thrombosis: a scoping review","authors":"Veronica DeYoung,&nbsp;Hassan Masood,&nbsp;Peter Andrisani,&nbsp;Arthane Kodeeswaran,&nbsp;Taylor Sparring,&nbsp;Jaskirat Arora,&nbsp;Natasha Savic,&nbsp;Jonathan L. Babulic,&nbsp;Patricia C. Liaw,&nbsp;Davide Matino,&nbsp;Alison Fox-Robichaud,&nbsp;Colin A. Kretz","doi":"10.1016/j.rpth.2026.106863","DOIUrl":"10.1016/j.rpth.2026.106863","url":null,"abstract":"<div><h3>Background</h3><div>von Willebrand factor (VWF) plays a significant role in blood coagulation and inflammation and is a key driver of many thrombotic conditions. Identifying appropriate mouse models is critical to developing effective and safe VWF-targeted therapies.</div></div><div><h3>Objectives</h3><div>This scoping review aims to identify and evaluate all mouse models of thrombosis that have demonstrated functional involvement of VWF and/or its primary regulator, ADAMTS-13, followed by a critical review of common models.</div></div><div><h3>Methods</h3><div>A comprehensive literature search was conducted to identify studies published from database inception to October 2024 that used mouse models of thrombosis. Two reviewers independently screened and extracted data on model methodology and outcomes, as well as secondary measures of thrombosis if they pertain directly to VWF or indirectly through ADAMTS-13.</div></div><div><h3>Results</h3><div>A total of 143 studies published between 2000 and 2024 were included; 32.6% models used C57BL/6 mice, and 32.7% used male mice only. Mice age and weight were reported in 79.7% and 30.8% of studies, respectively. Ferric chloride models were used in 45 (31.5%) studies, and laser injury models in 14 (9.8%). The most common systemic thrombosis model was thrombotic thrombocytopenic purpura (21 studies, 14.7%), and there were 16 other diseases studied with secondary thrombosis outcomes pertaining to VWF.</div></div><div><h3>Conclusion</h3><div>There is significant methodological heterogeneity across mouse models of thrombosis that have demonstrated involvement of VWF. Lack of standardization negatively impacts cross-study comparability and hinders the strength of preclinical studies of novel therapeutics. Standardization of model methodology is urgently needed, and in the future, researchers should consider the impact of methodology on VWF-related outcomes when designing their studies.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"10 7","pages":"Article 106863"},"PeriodicalIF":3.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149162563","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":"Assay or astray? discrepancies in efanesoctocog alfa monitoring","authors":"Agathe Herb,&nbsp;Jordan Wimmer,&nbsp;Sarra Derbal,&nbsp;Manon Dolt,&nbsp;Léa Pierre,&nbsp;Olivier Feugeas,&nbsp;Laurent Mauvieux,&nbsp;Dominique Desprez,&nbsp;Laurent Sattler","doi":"10.1016/j.rpth.2026.106994","DOIUrl":"10.1016/j.rpth.2026.106994","url":null,"abstract":"<div><h3>Background</h3><div>Efanesoctocog alfa (EFA) is an ultraextended half-life factor VIII (FVIII) with a unique structure that may influence laboratory monitoring. Although assay discrepancies have been described in spiked samples, data in patient samples are limited.</div></div><div><h3>Objectives</h3><div>To assess the performance of seven FVIII assays for measuring EFA in patient plasma and compare results with those from spiked samples.</div></div><div><h3>Methods</h3><div>FVIII activity was measured using four one-stage assays (OSAs) and three chromogenic assays (CSAs) on two analyzers. Assays were calibrated either with human plasma or an EFA-specific calibrator. Measurements were performed in FVIII-deficient plasma spiked with EFA and in samples from patients with hemophilia A treated with EFA. Results were compared with a reference assay (Actin FSL) and expressed as recovery percentages (acceptable range: 80%–120%).</div></div><div><h3>Results</h3><div>In spiked samples, plasma-calibrated CSA overestimated FVIII activity (+37% to +94%), whereas EFA-calibrated CSA showed good agreement with the reference assay (+2% to +16%). OSA results were generally acceptable, with slight underestimation depending on the reagent. In patient samples, patterns differed markedly: most assays deviated significantly from the reference, and none remained within the acceptable recovery range across the full FVIII spectrum. Compared with spiked samples, discrepancies were more pronounced at later time points and trough levels, where OSA tended to overestimate FVIII activity, whereas EFA-calibrated CSA underestimated activity.</div></div><div><h3>Conclusion</h3><div>Assay performance for EFA differs between spiked and patient samples, underscoring the limitations of <em>in vitro</em> evaluations. No assay provided consistent agreement with the reference assay across the full FVIII range, highlighting the need for cautious interpretation and improved standardization of EFA monitoring in clinical practice.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"10 7","pages":"Article 106994"},"PeriodicalIF":3.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149283035","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":"Unexplained recurrent diffuse bleeding diathesis as a manifestation of nonaneurysmal intimomedial mucoid degeneration","authors":"Cynthia Frima,&nbsp;Jan Jaap Spijkstra,&nbsp;Bernadette Schurink,&nbsp;Adriaan G. Holleboom","doi":"10.1016/j.rpth.2026.106978","DOIUrl":"10.1016/j.rpth.2026.106978","url":null,"abstract":"<div><h3>Background</h3><div>Intimomedial mucoid degeneration (IMMD) is an exceptionally rare and potentially life-threatening vasculopathy characterized by mucoid accumulation within the intima and media, destruction of elastic fibers, and subsequent arterial wall weakening. Previously reported cases have been associated with aneurysmal disease, often accompanied by an increased risk of intraoperative hemorrhage.</div></div><div><h3>Key Clinical Question</h3><div>Should IMMD be considered in patients with bleeding of unknown cause despite the absence of aneurysms?</div></div><div><h3>Clinical Approach</h3><div>We describe a patient with an isolated, recurrent, and unexplained severe diffuse bleeding diathesis in the absence of any detectable aneurysm. He ultimately died of multiorgan failure following profound hemorrhagic shock due to retroperitoneal bleeding. Postmortem histopathology revealed features consistent with IMMD despite the lack of aneurysmal changes.</div></div><div><h3>Conclusion</h3><div>This case highlights a broader clinical expression of IMMD than previously recognized by demonstrating that recurrent severe bleeding may occur in the absence of aneurysmal disease. It underscores the importance of further case reporting to improve future diagnosis and management.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"10 7","pages":"Article 106978"},"PeriodicalIF":3.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13691139/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149272656","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":"Association of the sarcopenia index based on the serum creatinine to cystatin C ratio and genetic risk with venous thromboembolism: a population-based cohort study","authors":"Yuyang Sun,&nbsp;Tingting Liu,&nbsp;Jun Deng,&nbsp;Xi Cao,&nbsp;Liang Tang,&nbsp;Xingjie Hao,&nbsp;Yu Hu","doi":"10.1016/j.rpth.2026.106973","DOIUrl":"10.1016/j.rpth.2026.106973","url":null,"abstract":"<div><h3>Background</h3><div>Venous thromboembolism (VTE) results from the interplay between environmental and genetic factors. The relationship between sarcopenia index (SI) and VTE risk remains unclear. The combined effects of SI and genetic susceptibility on VTE risk have not been fully elucidated. We therefore investigated these associations using data from the UK Biobank.</div></div><div><h3>Methods</h3><div>Cox proportional hazards models were used to assess the relationship between SI and genetic susceptibility with incident VTE. Restricted cubic spline models were applied to examine the exposure-response relationships.</div></div><div><h3>Results</h3><div>Among 447,681 participants, 12,008 developed VTE during a median follow-up of 13.4 years. Higher SI was associated with a lower risk of VTE. The fully adjusted hazard ratios (HRs) for Q2 to Q4 vs Q1 were 0.88 (95% CI, 0.83-0.92), 0.82 (95% CI, 0.78-0.87), and 0.77 (95% CI, 0.72-0.82), respectively. Restricted cubic spline analysis showed an inverse nonlinear association between SI and VTE (<em>P</em> for nonlinear &lt;.001). Participants with high genetic risk had a higher risk of VTE (HR = 2.56; 95% CI, 2.42-2.72). Furthermore, participants with both low SI levels and high genetic susceptibility exhibited a significantly increased risk of VTE compared with those with high SI and low genetic risk (HR = 3.60; 95% CI, 3.14-4.12).</div></div><div><h3>Conclusion</h3><div>Participants with lower SI had a significantly higher risk of VTE. Consistent with previous UK Biobank findings, higher polygenic risk score levels were associated with an increased risk of VTE. Moreover, participants with both low SI levels and high genetic risk exhibited the greatest risk of VTE. These findings suggest that SI may serve as a simple and accessible marker for identifying individuals at elevated risk of VTE.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"10 7","pages":"Article 106973"},"PeriodicalIF":3.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149162565","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":"Reevaluating hypercoagulability in the portal vein and the impact of transjugular intrahepatic portosystemic shunt on platelet activation in cirrhosis","authors":"Viktoria Möller,&nbsp;Anselm Kunstein,&nbsp;Christian Weigel,&nbsp;Stephanie D. Wolf,&nbsp;Alexander Killer,&nbsp;Matthias Dille,&nbsp;Mihael Vucur,&nbsp;Kai Jannusch,&nbsp;Eric Tietz,&nbsp;Tom Luedde,&nbsp;Peter Minko,&nbsp;Johannes G. Bode,&nbsp;Carolin Angendohr","doi":"10.1016/j.rpth.2026.106977","DOIUrl":"10.1016/j.rpth.2026.106977","url":null,"abstract":"<div><h3>Background</h3><div>In liver cirrhosis, dysregulated hemostasis predisposes patients to both hemorrhagic and thrombotic complications. The portal vein (PV) is a frequent site of thrombosis, but the underlying mechanisms remain incompletely understood. Whether the PV constitutes a locally hypercoagulable environment remains controversial, as previous studies have reported conflicting findings regarding portal venous platelet activation, endothelial dysfunction, and coagulation factor alterations.</div></div><div><h3>Methods</h3><div>We enrolled 34 patients with liver cirrhosis undergoing transjugular intrahepatic portosystemic shunt (TIPS) implantation. Platelet count, activation, and reactivity were assessed by flow cytometry before and after TIPS, as well as across multiple vascular compartments, including the PV, superior mesenteric vein, hepatic vein, and peripheral vein. In addition, we measured key coagulation factors involved in secondary hemostasis in the indicated vessels.</div></div><div><h3>Results</h3><div>Platelet counts declined with advancing liver disease, but no changes in platelet activation or reactivity were observed. Despite elevated platelet counts in the PV, we found no evidence of increased platelet activation or reactivity. Moreover, no compartment-specific differences were detected in the selected conventional coagulation parameters assessed, except for lower fibrinogen levels in the PV compared with the hepatic vein. TIPS implantation did not lead to a short-term increase in platelet activation.</div></div><div><h3>Conclusions</h3><div>In this cohort, we found no evidence of increased portal venous platelet activation or reactivity. Apart from lower fibrinogen levels in the PV compared with the hepatic vein, the selected conventional coagulation parameters assessed did not show consistent compartment-specific differences. These findings do not support the concept of a PV-specific hypercoagulable state as assessed by the present platelet activation and reactivity measures and selected coagulation assays. Rather, they suggest that relief of portal hypertension and reduction of venous stasis may be more relevant for thrombosis risk than changes in platelet activation alone.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"10 7","pages":"Article 106977"},"PeriodicalIF":3.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149213332","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}