Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis最新文献

{"title":"Efficacy of Factor Xa Inhibitors Versus Placebo in Thromboprophylaxis for Cancer-Associated Thromboembolism: A Systematic Review and Meta-analysis.","authors":"Amna Kamil, Sandhiya Prem Kumar, Rumaisa Zulfiqar, Eiman Araib, Bibi Samia Khan, Muhammad Saad Khan, Muhammad Mohsin Khan, Umaimah Naeem, Aminath Waafira","doi":"10.1177/10760296251372947","DOIUrl":"10.1177/10760296251372947","url":null,"abstract":"<p><p>BackgroundCancer patients are at significantly increased risk of venous thromboembolism (VTE), a leading cause of morbidity and mortality in this population. While traditional anticoagulants like low-molecular-weight heparin (LMWH) and vitamin K antagonists (VKAs) are commonly used, their limitations have prompted growing interest in direct oral anticoagulants (DOACs), particularly Factor Xa inhibitors. However, concerns about bleeding risks persist. This meta-analysis aims to evaluate the efficacy of Factor Xa inhibitors versus placebo for thromboprophylaxis in cancer-associated VTE.MethodsThis systematic review and meta-analysis followed PRISMA guidelines and was registered with PROSPERO (CRD42024574869). A comprehensive search of PubMed, Cochrane Library, Scopus, Google Scholar, and ClinicalTrials.gov was conducted without language or demographic restrictions. Data were extracted independently by two reviewers and analyzed using a random-effects model.ResultsSix RCTs with a total of 2330 participants met the inclusion criteria. The pooled analysis showed a non-significant reduction in VTE incidence with Factor Xa inhibitors (RR = 0.33, 95% CI: 0.05-2.10, <i>P</i> = .24). However, there was a nearly twofold increased risk of major bleeding (RR = 1.90, 95% CI: 1.00-3.62, <i>P</i> = .05). No significant effect was found for clinically relevant non-major bleeding (CRNMB) (RR = 1.28, 95% CI: 0.73-2.22, <i>P</i> = .39).ConclusionFactor Xa inhibitors may reduce the risk of VTE in cancer patients but appear to increase the risk of major bleeding. The evidence remains inconclusive due to limited event numbers and wide confidence intervals, highlighting the need for larger trials to better assess safety and efficacy.</p>","PeriodicalId":520590,"journal":{"name":"Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis","volume":"31 ","pages":"10760296251372947"},"PeriodicalIF":2.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12413517/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145002745","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Recent Advances in Gene Therapy for Hemophilia.","authors":"Xiaojuan Pang, Jinxian Fu, Zhongqi Zhou, Nannan Tang, Cia-Hin Lau, Yingfei Wen, Ping Chen, Jing Zhao, Hongman Xue","doi":"10.1177/10760296251378455","DOIUrl":"10.1177/10760296251378455","url":null,"abstract":"<p><p>Hemophilia, an X-linked monogenic disorder, arises from mutations in the <i>F8</i> or <i>F9</i> genes, which encode clotting factor VIII (FVIII) or clotting factor IX (FIX), respectively. As a prominent hereditary coagulation disorder, hemophilia is clinically manifested by spontaneous hemorrhagic episodes. Severe cases may progress to complications such as stroke and arthropathy, significantly compromising patients' quality of life. Hemophilia has a monogenic nature, coupled with quantifiable therapeutic endpoints and predictable treatment outcomes. These characteristics render it an ideal candidate for gene therapy studies. Currently, Food and Drug Administration (FDA)-approved gene therapies utilize recombinant adeno-associated virus (AAV) vectors to deliver functional transgene cassettes to hepatocytes. These therapies offer distinct advantages: a single intravenous administration achieves sustained FVIII and FIX activity levels, providing robust hemostatic control while markedly enhancing patients' quality of life. However, several challenges remain, including immunogenicity, thrombotic risks, potential gene integration, and prohibitive costs. Future endeavors should prioritize expanding patient eligibility and integrating precision gene-editing technologies to mitigate these limitations. In this review, we provide a comprehensive overview of recent advances and emerging strategies in hemophilia gene therapy, with a particular focus on clinical translation and technological innovation. Ongoing research in this field remains pivotal to overcome existing barriers, enhance treatment accessibility, and ultimately realize curative potential for patients with hemophilia.</p>","PeriodicalId":520590,"journal":{"name":"Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis","volume":"31 ","pages":"10760296251378455"},"PeriodicalIF":2.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12423543/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145031618","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Coagulation Biomarkers and Mortality in Severe COVID-19: The Prognostic Value of Factor XIII Decline and Elevated D-Dimers.","authors":"Christopher Cibis, Elisabeth Hannah Adam, Florian Gatzke, Steffen Rauchfuss, Stefanie Roth, Wolfgang Miesbach","doi":"10.1177/10760296251364266","DOIUrl":"10.1177/10760296251364266","url":null,"abstract":"<p><p>IntroductionThis study investigated the roles of d-dimers, fibrinogen, activated partial thromboplastin time (aPTT), and Factor XIII (FXIII) in critically ill COVID-19 patients.Methods68 patients were included into the study based on the inclusion criteria. Severe illness was defined as COVID-19 pneumonia leading to acute respiratory failure requiring mechanical ventilation and ICU (Intensive Care Unit) care. Patients who received Extracorporeal Membrane Oxygenation therapy were excluded due to its effects on the coagulation system. Blood samples were collected on day one and days five to seven, to measure coagulation parameters (aPTT, d-dimer, fibrinogen, and FXIII).ResultsIn total, 59% of the patients died during their hospital stay. Coagulation parameters showed elevated d-dimer levels in 95.6% of patients, while FXIII activity significantly decreased during the first week in ICU. A drop in FXIII activity over the first week (FXIII Δ) emerged as a significant predictor of in-hospital mortality (HR = 2.174, <i>P</i> = .031), while d-dimers did not. No significant changes in aPTT, fibrinogen, or d-dimer levels were observed between days one and five to seven.ConclusionThe change in Factor XIII activity during the first week in ICU (FXIII Δ) emerged as an independent predictor of in-hospital mortality. D-dimer levels were elevated in nearly all patients, but did not serve as an independent predictor of in-hospital mortality, possibly due to the homogeneity of the cohort. Overall, the results emphasize the importance of monitoring d-dimers and Factor XIII in predicting disease severity and outcomes in COVID-19 patients.</p>","PeriodicalId":520590,"journal":{"name":"Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis","volume":"31 ","pages":"10760296251364266"},"PeriodicalIF":2.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12446857/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145083263","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Abelacimab for Acute Pulmonary Embolism: Translating Dual FXI/XIa Inhibition from Mechanism to Clinical Prospects.","authors":"Mengdie Luo, Jun Wu","doi":"10.1177/10760296251386036","DOIUrl":"10.1177/10760296251386036","url":null,"abstract":"<p><p>Acute pulmonary embolism (PE) remains a life-threatening condition. A critical therapeutic dilemma exists for a significant proportion of patients at high bleeding risk（approximately 30%-50%）. For these patients, anticoagulation is the mainstay, but the use of conventional agents (warfarin, DOACs, heparins) is severely limited by the risk of major bleeding, creating a substantial unmet medical need. Abelacimab addresses this unmet need through a novel mechanism: dual inhibition of factor XI (FXI) and activated FXI (FXIa). This paradigm shift uncouples potent antithrombotic efficacy from hemostasis, selectively suppressing contact pathway-driven thrombosis while preserving tissue factor-mediated hemostasis. We synthesize its unique mechanism of action, pharmacokinetic profile, and existing clinical evidence to evaluate its role in addressing this clinical gap. Abelacimab's mechanism selectively inhibits the contact activation pathway-crucially overactive in the RBC-rich thrombi characteristic of PE-while preserving tissue factor-mediated physiological hemostasis. Clinically, abelacimab demonstrated significantly lower rates of major bleeding or clinically relevant non-major bleeding compared to rivaroxaban in atrial fibrillation prophylaxis (AZALEA-TIMI 71 trial) and to enoxaparin in post-arthroplasty venous thromboembolism prevention (ANT-005 trial), establishing a favorable clinical safety profile. Its favorable pharmacokinetics (long half-life supporting monthly subcutaneous administration, with minimal renal/hepatic clearance) further simplify management and enhance its suitability for patients with PE. However, current evidence is extrapolated from AF/VTE prophylaxis studies, lacking direct human data for acute PE management. Urgent phase III trials (eg, PE-FOCUS) are therefore essential to validate efficacy and safety in acute PE, potentially redefining the anticoagulation paradigm.</p>","PeriodicalId":520590,"journal":{"name":"Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis","volume":"31 ","pages":"10760296251386036"},"PeriodicalIF":2.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12501462/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145240775","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pharmacists' Knowledge of Abnormal Uterine Bleeding in Women on Anticoagulant Therapy: A French Survey.","authors":"Sibylle Frerebeau, Benjamin Planquette, Gabrielle Sarlon, Nicolas Gendron","doi":"10.1177/10760296251350078","DOIUrl":"10.1177/10760296251350078","url":null,"abstract":"<p><p>ObjectivesThis study aimed to assess the role of community pharmacists in managing abnormal uterine bleeding (AUB) in women on anticoagulant therapy, particularly in the context of a physician shortage in France, which exacerbates the pharmacist's role. Recognizing these bleedings, which impair the quality of life for women, is an essential role of pharmacists and could be crucial in preventing the risk of discontinuing anticoagulant treatment.MethodsWe conducted an anonymous survey from December 2023 to January 2024 among pharmacists in France to explore their experiences with patients on anticoagulants who presented at pharmacies with questions about AUB.ResultsOf the 115 respondents, only 9 (7.8%) pharmacists had been approached by patients with questions about AUB while on anticoagulants. These pharmacists reported a majority of cases in women of reproductive age, the drug the most frequently cited was rivaroxaban. No significant demographic differences were observed between the pharmacists who had faced these questions and those who had not. Additionally, 99 (86.1%) pharmacists considered that they did not have enough skills to adequately advise female patients about abnormal uterine/genital bleeding under anticoagulant therapy.ConclusionOur study showed that community pharmacists are not the primary healthcare contact for patients with AUB under anticoagulant therapy. Despite this, the low frequency of pharmacist-patient interactions regarding AUB suggests potential under-diagnosis and under-communication of adverse effects that negatively impact the quality of life of women on anticoagulants.</p>","PeriodicalId":520590,"journal":{"name":"Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis","volume":"31 ","pages":"10760296251350078"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12174673/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144311187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Establishment Trimester-Specific Reference Intervals of Anticoagulation and Fibrinolysis Indicators in Healthy Pregnant Women in Beijing, China.","authors":"Linzi Miao, Xiao Sun, Zijing Zhu, Yuanyuan Li, Yao Lu, Ran You, Chenxue Qu","doi":"10.1177/10760296251342456","DOIUrl":"10.1177/10760296251342456","url":null,"abstract":"<p><p>ObjectiveContinuously tracking and monitoring the changes in indicators of anticoagulation and fibrinolysis in healthy pregnant women during different pregnancy periods, to establish trimester-specific reference intervals.MethodsA total of 200 healthy pregnant women registered in Peking University First Hospital were enrolled. Venous blood samples were collected at different pregnancy periods, and thirteen related anticoagulation and fibrinolysis indicators were tested. The impact of pregnancy periods on these indicators were analyzed, and corresponding reference intervals were established.ResultsSignificant differences were observed among the non-pregnant group and the first trimester(6-8weeks), second trimester(24-28weeks), and third trimester(36-38weeks) groups for antithrombin (AT), protein S activity (PS Ac), free protein S (FPS), protein C (PC), D-Dimer, fibrin/fibrinogen degradation products (FDP), plasmin inhibitor (PI), plasminogen (PLG), von Willebrand factor activity (vWF:Ac), von Willebrand factor antigen (vWF:Ag), lupus anticoagulant. AT, PS Ac, and FPS showed a downward trend with increasing gestational period. PI was significantly increased in the first trimester. PLG and vWF:Ag was higher in the second trimester and the third trimester. vWF:Ac, D-Dimer and FDP showed an continuously upward trend with increasing gestational period; the normalized SCT ratio (SCT TR)was significantly lower in the third trimester; the normalized dRVVT ratio (dRVVT TR)was significantly higher in the second and third trimester.ConclusionThis study established reference intervals for thirteen anticoagulant and fibrinolytic indicators in healthy pregnant women in Beijing, China. Additionally, pregnancy periods affect the test results of anticoagulant and fibrinolytic indicators in pregnant women, and the reference intervals should be established separately according to pregnancy periods.</p>","PeriodicalId":520590,"journal":{"name":"Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis","volume":"31 ","pages":"10760296251342456"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12174712/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144304355","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of Early Versus Delayed DOACs Initiation in Acute Ischemic Stroke Patients with AF.","authors":"Zhenglei Yu, Wengen Zhu","doi":"10.1177/10760296251375864","DOIUrl":"10.1177/10760296251375864","url":null,"abstract":"<p><p>The optimal timing for initiating direct oral anticoagulants (DOACs) in patients with atrial fibrillation (AF) following acute ischemic stroke (AIS) remains a critical clinical question. This review evaluates findings from three pivotal randomized controlled trials-TIMING, ELAN, and OPTIMAS-comparing early versus delayed DOAC initiation in AF patients with AIS. Collectively, these trials provide strong evidence supporting the safety and efficacy of early initiation of DOACs in patients with AIS and AF. The TIMING trial demonstrated the non-inferiority of early DOAC initiation (≤4 days) compared to delayed initiation (5-10 days), with numerically lower rates of ischemic stroke and no cases of symptomatic ICH. The ELAN trial further corroborated these findings, showing no significant difference in the composite outcome of recurrent ischemic stroke, systemic embolism, or major hemorrhage between early initiation (≤48 h for minor/moderate strokes; days 6-7 for major strokes) and later initiation (days 3-4 to 12-14). OPTIMAS, the largest trial to date, confirmed the non-inferiority of early initiation (≤4 days) versus delayed initiation (7-14 days), without an increased risk of symptomatic ICH and with comparable rates of recurrent ischemic events.</p>","PeriodicalId":520590,"journal":{"name":"Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis","volume":"31 ","pages":"10760296251375864"},"PeriodicalIF":2.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12409021/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144986070","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Linking Cortical Structure and Cerebrospinal Fluid Metabolites with Venous Thromboembolism Risk: A Two-Step Mendelian Randomization and Co-Localization Analysis.","authors":"Tianni Liu, Junxian Li, Qiong Liu, Guojun Liang","doi":"10.1177/10760296251360018","DOIUrl":"10.1177/10760296251360018","url":null,"abstract":"<p><p>BackgroundVenous thromboembolism (VTE) is a complex vascular disorder, and emerging research suggests potential neurovascular and metabolic factors in its pathogenesis. However, the specific roles of cortical structure features and cerebrospinal fluid (CSF) metabolites in VTE risk remain underexplored.MethodsUsing Mendelian randomization (MR) analysis, we examined associations between cortical features, CSF metabolites and VTE. Mediation MR and co-localization analyses were employed to explore genetic pathways and potential mediatory effects.ResultsMR analysis initially identified associations between six cortical features and fifteen CSF metabolites with VTE. After false discovery rate (FDR) correction, lingual gyrus thickness remained statistically significant, while isoleucine and methylmalonate showed suggestive associations. Mediation MR analysis revealed no causal relationship between lingual gyrus thickness and the CSF metabolites isoleucine or methylmalonate. Co-localization analysis indicated low posterior probabilities for shared genetic variants, suggesting that these traits influence venous thromboembolism (VTE) through distinct biological mechanisms.ConclusionThe findings suggest that MRI-based cortical structure features and CSF profiling hold potential as complementary tools for assessing VTE risk. Further research is warranted to investigate the neurovascular and metabolic mechanisms underlying VTE.</p>","PeriodicalId":520590,"journal":{"name":"Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis","volume":"31 ","pages":"10760296251360018"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12276481/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144661730","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy and Safety of Prothrombin complex Concentrate in Patients with Massive Intraoperative Bleeding During non-Cardiac Surgery: A Retrospective Cohort Study.","authors":"Bo Tang, Yuelun Zhang, Jia Gan, Lulu Ma, Yuguang Huang","doi":"10.1177/10760296251356202","DOIUrl":"10.1177/10760296251356202","url":null,"abstract":"<p><p>PurposeThe efficacy and safety of four-factor prothrombin complex concentrate (4F-PCC) in managing bleeding during non-cardiac surgery are unclear. We investigated the associations of 4F-PCC with postoperative RBC transfusion and adverse events in non-cardiac surgery patients with massive intraoperative bleeding.MethodsThis retrospective cohort study consecutively included non-cardiac surgery patients with massive intraoperative bleeding at a tertiary hospital (2014-2020). Administration of 4F-PCC was categorized into dose groups based on quartiles: 0 (reference), 2.8-6.7, 6.7-11.5, 11.5-19.4, and 19.4-87.5 IU/kg. Outcomes included postoperative RBC transfusion, major thromboembolic events, severe acute kidney injury, and lengths of ICU and hospital stay.ResultsOf 137 patients, 89 (65.0%) received 4F-PCC. The 6.7-11.5 IU/kg group were significantly associated with reduced postoperative RBC transfusion compared to the non-4F-PCC group (adjusted mean difference, -1.29 units; 95%CI, -2.55 to -0.04 units, <i>P</i> = 0.044). Such findings were not observed in other dose groups. Notably, the benefits were particularly significant in patients with preoperative platelet count ≥150 × 109/l (<i>P</i> = 0.031), and fibrinogen ≥3 g/l (<i>P</i> = 0.025). The 6.7-11.5 IU/kg group exhibited comparable incidences of major thromboembolic events (13.0% vs 10.4%) and severe acute kidney injury (8.7% vs 8.3%) compared to the non-4F-PCC group. The lengths of ICU and hospital stay were similar across groups.Conclusion4F-PCC may be associated with decreased postoperative RBC transfusion in non-cardiac surgery patients experiencing massive intraoperative bleeding, without a significant increase in the risk of major thromboembolic events. Randomized trials with stratified dosing are warranted to confirm efficacy, safety, and determine optimal doses.Clinical Trial NumberChiCTR2500096573.</p>","PeriodicalId":520590,"journal":{"name":"Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis","volume":"31 ","pages":"10760296251356202"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12227943/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144556480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Coagulation and Fibrinolysis Dysregulation in β-Thalassemia Major: Potential Impact of Splenectomy and Medications on Thrombotic Risk.","authors":"Ola M Al-Sanabra, Manal A Abbas, Wafà J Hazà, Abeer A Hazàa, Ahmad K Al Tibi","doi":"10.1177/10760296251359291","DOIUrl":"10.1177/10760296251359291","url":null,"abstract":"<p><p>ObjectiveThe dysregulation of clotting factors in β-thalassemia major is not fully understood. This case-control study examines the impact of splenectomy and medications on clotting factor dysregulation.MethodsLab. tests of coagulation and fibrinolysis were performed.ResultsThis study included 60 β-thalassemia major patients of both sexes (7-35 years) and 20 age-matched controls. None of the participants had a previous thrombotic event. No difference existed between control and β-thalassemia groups in all tested parameters except for protein S (PS) which was 26.1% lower in β-thalassemia group compared to the control group (p = 0.0001) while D-Dimer, plasminogen activator inhibitor-1 (PAI-1) and platelet count showed an increase in their levels by 53.6%, 93.4% and 112.7%, respectively compared to the control (p = 0.004, p = 0.0001, p = 0.0001, respectively). Notably, a strong positive correlation existed between platelet count and PAI-1 (r = 0.669, p = 0.0001) in β-thalassemia group. Splenectomized patients had higher platelet count (+45.2%), PAI-1 (+98.1%), fibrinogen (+18.9%) and tPA (+197.2%) compared to the non-splenectomized group (all p < 0.05). No significant differences in PS, D-dimer, PT, INR, aPTT, fibrinogen, PC, TF, tPA or ADAMTS13 were found between β-thalassemia major patients taking and not taking aspirin. However, a higher platelet count (+37.1%) and PAI-1 level (+58.9%), along with a lower vWF level (-25.6%), were observed between these two groups (all p < 0.05).ConclusionElevation in PAI-1 and platelet count in splenectomized β-thalassemia major could increase the risk of having a thrombotic event. Medications may have significant interactions with blood coagulation in β-thalassemia.</p>","PeriodicalId":520590,"journal":{"name":"Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis","volume":"31 ","pages":"10760296251359291"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12264414/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144639566","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}