Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis最新文献

{"title":"Outcomes of recombinant activated factor VIIa (NovoSeven) therapy in glanzmann thrombasthenia: two case reports.","authors":"Eman Almatar, Sondus Alsharidah, Omnia A Hashem","doi":"10.1097/MBC.0000000000001380","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001380","url":null,"abstract":"<p><p>Glanzmann thrombasthenia (GT) is a rare autosomal recessive platelet function disorder resulting from qualitative or quantitative defects in the GPIIb/IIIa (integrin αIIbβ3) complex. Patients typically present with mucocutaneous bleeding. Standard treatment involves antifibrinolytic agents and platelet transfusions; however, repeated transfusions may lead to alloimmunization and platelet refractoriness. Recombinant activated factor VIIa (rFVIIa; NovoSeven) offers an alternative hemostatic approach. We present two pediatric cases of GT managed with rFVIIa. The first case involves a 12-year-old female with a strong family history of GT, who initially responded well to rFVIIa prophylaxis but later experienced increased bleeding episodes during puberty. The second case is a 20-year-old male with a history of mucocutaneous bleeding, who achieved long-term bleeding control on regular rFVIIa prophylaxis. Both patients demonstrated reduced bleeding frequency and improved quality of life without thromboembolic complications. These cases underscore the therapeutic potential and safety of rFVIIa in managing bleeding episodes and preventing alloimmunization in GT, particularly when platelet transfusions are ineffective or contraindicated, and highlight the importance of individualized treatment approaches and the need for further research to refine prophylactic regimens in GT.</p>","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144651988","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Platelet aggregation response in children with infantile haemangioma under propranolol treatment.","authors":"Havva Özüm Kolsuz, Zeynep Canan Özdemir, Özcan Bör","doi":"10.1097/MBC.0000000000001383","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001383","url":null,"abstract":"<p><p>Infantile haemangioma is characterized by the proliferation of immature endothelial cells. A small portion requires treatment. Beta blockers are a good treatment option; however, it has been shown that beta blockers cause a decrease in the aggregation response of platelets. Twenty healthy children and 20 children with a diagnosis of infantile haemangioma who were started on propranolol treatment were included in the study. All patients were given propranolol 1 mg/kg/day in two doses. Haemangioma size, appearance features, blood count parameters and percentage of the maximal amplitude of platelets were compared before treatment and at the first month of treatment. The percentage of the maximal amplitude was determined using the light transmission aggregometry method. A total of 23 haemangiomas were detected. The mean size of haemangioma at the first month after treatment (2.46 cm) was statistically significantly lower than before treatment (2.65 cm) (P < 0.05). A lighter shade was noticeable in six of 23 (26.1%) haemangiomas. The number of patients whose swelling decreased (13 patients, 68.4%) was significantly greater than the number whose swelling did not decrease (six patients, 31.6%) (P < 0.05). There was no difference in blood count parameters and percentage of the maximal amplitude of platelets between groups (P > 0.05, for all). When 1 mg/kg/day propranolol was given to children with infantile haemangioma, it was shown that propranolol was effective in the response to treatment even after 1 month of treatment, and there was no change in the blood count parameters and platelet aggregation response.</p>","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693045","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rosa Centifolia L. effect on hemostasis: a multifaceted study combining in-vitro, in-vivo, and in-silico investigations of molecular mechanisms.","authors":"Omayma El Meziouy, Moulay Hfid Youssoufi, Ali Berraaouan, Fatima Zahra Lafdil, Sergey Shityakov, Abdelkhaleq Legssyer, Abderrahim Ziyyat, Khalil Azzaoui, Hassane Mekhfi","doi":"10.1097/MBC.0000000000001379","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001379","url":null,"abstract":"<p><p>Thrombus formation is a major cause of cardiovascular disease-related death. Traditional medicine has long utilized plants for therapeutic purposes. The aqueous extract of Rosa centifolia L., commonly used in Moroccan folk medicine, was investigated for its potential anticoagulant and antioxidant properties. The study assessed platelet aggregation inhibition, bleeding time, antioxidant activity, and coagulation parameters, including activated partial thromboplastin time (aPTT), prothrombin time (PT), and fibrinogen concentration. Platelet aggregation was induced using thrombin, ADP, arachidonic acid, and collagen. Antioxidant activity was evaluated using the FRAP and β-carotene assays. Phytochemical analysis identified the presence of phenols, flavonoids, and tannins. Additionally, computational molecular docking was performed to analyze the interaction between prostaglandin G/H synthase 1 (COX-1) and kaempferol. The aqueous extract significantly inhibited platelet aggregation and prolonged bleeding time, particularly at 1 mg/ml, where it also extended aPTT. Antioxidant activity was confirmed through FRAP and β-carotene assays. Computational docking of kaempferol with COX-1 generated 10 binding modes, demonstrating favorable binding affinities and validating the docking protocol using the co-crystallized diclofenac structure. The findings suggest that Rosa centifolia L. exhibits anticoagulant and antioxidant properties, supporting its potential therapeutic application in preventing oxidative stress and thrombosis-related disorders.</p>","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144629297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Silent progression of hemophilic arthropathy of the elbow: case reports of three pediatric patients.","authors":"Atsuki Yamashita, Mika Mori, Yotaro Umezawa, Tomoko Ashikaga, Chiai Nagae, Ryota Koyama, Takeshi Arai, Naoki Haraguchi, Yuki Saito, Atsuko Fujikawa, Masashi Taki","doi":"10.1097/MBC.0000000000001382","DOIUrl":"10.1097/MBC.0000000000001382","url":null,"abstract":"<p><p>We present three pediatric cases of hemophilic arthropathy (HA) of the elbow that had already progressed to advanced stage with osteochondral lesions at the time of diagnosis-Case 1: 13-year-old boy, severe hemophilia A; Case 2: 13-year-old boy, moderate hemophilia A; Case 3: 15-year-old boy, severe hemophilia A. HA results from repeated hemarthrosis. Notably, Cases 2 and 3 had no history of clinically recognized elbow hemarthrosis before HA diagnosis, and Case 1 had only two episodes of hemarthrosis. Cases 2 and 3 did not report elbow extension restriction and pain for 1 or 2 years, as their daily lives were minimally affected. Since full elbow extension or flexion is rarely needed in daily life, and restricted elbow motion can be compensated by other motions, leading to less inconvenience. Consequently, HA of the elbow often remains unnoticed until it progresses to advanced stage; which necessitates careful monitoring from childhood.</p>","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144629298","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Safety and efficacy of thrombolytic interventions in the treatment of intermediate and high-risk pulmonary embolism.","authors":"Logan D Johnson, J Chase Cole, Michael J Erdman, Leela Krishna Teja Boppana, Nimeh Najjar, Elizabeth A Shald, Kirsten V Busey, Kelsey L Stilley, Jason A Ferreira","doi":"10.1097/MBC.0000000000001375","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001375","url":null,"abstract":"<p><strong>Background: </strong>Intermediate to high-risk pulmonary embolism management is controversial in the absence of hemodynamic instability. Anticoagulation with systemic thrombolysis is commonly employed in these patients, and alternative interventional therapies when contraindications to thrombolysis are present. No comparative evidence exists between systemic alteplase, tenecteplase and catheter directed thrombolysis (CDT) and practice continues to be clouded by this lack of literature.</p><p><strong>Research question: </strong>Does systemic alteplase, tenecteplase or CDT have better safety or efficacy outcomes in the treatment of intermediate to high-risk pulmonary embolism?</p><p><strong>Study design and methods: </strong>This was a retrospective, multicenter study including patients 18-90 years old with an acute presentation of intermediate-high risk PE receiving systemic or directed thrombolysis with therapeutic anticoagulation. The primary outcome was major bleeding complications within 72 h of treatment. Secondary outcomes included minor bleeding, hemodynamic decompensation, 28-day ventilator-free days, mortality and length of stay.</p><p><strong>Results: </strong>The primary outcome was significantly higher with tenecteplase and US-CDT when compared to alteplase (alteplase 10.9% vs. tenecteplase 31.1%, P = 0.004; US-CDT 21.4%, P = 0.03). After propensity matching, tenecteplase was associated with a higher rate of major bleeding (P = 0.13) while US-CDT showed a significantly higher risk of complications (P = 0.04). This was supported by multivariate logistic regression with alteplase vs. US-CDT (odds ratio 0.07; 95% confidence interval 0.01, 0.3). Tenecteplase receipt was not associated with different rates of hemodynamic decompensation or mortality compared to alteplase while US-CDT had significantly lower rates, likely secondary to acuity of illness.</p><p><strong>Interpretation: </strong>Alteplase was associated with the lowest risk of major bleeding, but with variable disease severities. Selection of agent for the treatment of PE continues to require analysis of patient-specific hemodynamics and disease severity.</p>","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144586164","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Current epidemiology of deaths for disseminated intravascular coagulation.","authors":"Camilla Mattiuzzi, Giuseppe Lippi","doi":"10.1097/MBC.0000000000001369","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001369","url":null,"abstract":"","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":"36 5","pages":"229-231"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144556474","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hereditary hepatic fibrinogen storage disease with a novel fibrinogen variant FGG c.1113T>A ( fibrinogen Seoul III ).","authors":"Kyong Eun Choi, Seon Young Kim, Hansol Kim, Yoon Zi Kim, Sang Yun Ha, Yon Ho Choe, Boram Kim, Hee-Jin Kim, Mi Jin Kim","doi":"10.1097/MBC.0000000000001358","DOIUrl":"10.1097/MBC.0000000000001358","url":null,"abstract":"<p><p>Hereditary hepatic fibrinogen storage disease (HHFS) is an extremely rare genetic disorder caused by mutations in the fibrinogen gamma ( FGG ) gene. Clinical presentations range from asymptomatic cases to severe symptoms. We present the case of a 9-year-old boy with persistently elevated liver enzymes who exhibited no clinical symptoms. A liver biopsy revealed hepatic fibrinogen storage disease, and genetic testing identified a heterozygous mutation, c.1113T>A, p.(Asn371Lys) in FGG . This is the first reported case of HHFS in Korea, as well as the first report of this novel mutation. Multigene panel testing played a crucial role in diagnosing this rare condition. Following three months of ursodeoxycholic acid medication, liver enzyme levels normalized, and the patient is currently under follow-up.</p>","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":"204-207"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144277969","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The dilemma of assessing hyperfibrinolysis through viscoelastic tests.","authors":"Rodolpho Augusto de Moura Pedro, Luiz Marcelo Sá Malbouisson","doi":"10.1097/MBC.0000000000001345","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001345","url":null,"abstract":"","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":"36 5","pages":"228-229"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144556475","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The prevalence and associated factors of aspirin resistance in JAK2V617F-mutated essential thrombocythemia and polycythemia vera.","authors":"Prachatip Phollarp, Wiyada Dankai, Ornkamon Wongtakan, Piangrawee Niprapan, Teerachat Punnachet, Nonthakorn Hantrakun, Pokpong Piriyakhuntorn, Thanawat Rattanathammethee, Sasinee Hantrakool, Chatree Chai-Adisaksopha, Adisak Tantiworawit, Lalita Norasetthada, Suree Lekawanvijit, Ekarat Rattarittamrong","doi":"10.1097/MBC.0000000000001376","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001376","url":null,"abstract":"<p><p>The primary aim was to determine the prevalence of aspirin resistance in JAK2V617F-mutated essential thrombocythemia and polycythemia vera patients. Secondary objectives included analyzing risk factors for aspirin resistance including JAK2V617F allele burden. This cross-sectional study included patients aged at least 18 years with JAK2V617F-mutated essential thrombocythemia and polycythemia vera who were prescribed aspirin. Blood samples were taken to measure JAK2V617F allele burden and serum thromboxane B2 (TXB2). Aspirin resistance was defined by serum TXB2 levels greater than 570 pg/ml. Clinical characteristics, laboratory data, JAK2V617F allele burden, and treatment were analyzed to identify risk factors. Seventy-eight patients were enrolled, with 27 (34.6%) having essential thrombocythemia and 51 (65.4%) having polycythemia vera. The prevalence of aspirin resistance was 100%. Factors associated with high TXB2 levels included a history of stroke [95% confidence interval (CI): 1.11-12.34; P = 0.034], hydroxyurea dose at least 15 mg/kg/day (95% CI: 1.04-8.93; P = 0.043), and platelet count at least 400 000 cells/μl (95% CI: 1.75-18.86; P = 0.004). JAK2V617F allele burden was not significantly correlated with high serum TXB2 (r2 = 0.087, P = 0.45). The prevalence of aspirin resistance was 100% in JAK2V617F-mutated polycythemia vera and essential thrombocythemia patients, based on serum TXB2 levels greater than 570 pg/ml. A history of stroke, hydroxyurea dose at least 15 mg/kg/day, and platelet count at least 400 000 cells/μl were associated with high TXB2 levels.</p>","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144510293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Whole blood thrombin generation hypercoagulable profile in a patient with hemolytic crisis due to paroxysmal nocturnal hemoglobinuria: a case report.","authors":"Angela Napolitano, Luca Spiezia, Cristiana Bulato, Elena Campello, Paolo Simioni","doi":"10.1097/MBC.0000000000001377","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001377","url":null,"abstract":"<p><p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematological disorder characterized by intravascular hemolysis, bone marrow failure, and increased thrombotic risk. Previous studies using plasma thrombin generation tests in PNH patients yielded conflicting results. Given the central role of cellular blood components in PNH, we hypothesized that whole blood thrombin generation (WB-TG) may provide a more comprehensive assessment of patients' coagulation profile. We report the case of a 25-year-old woman with PNH and admitted to Padova University Hospital for hemolytic crisis following an influenza A virus infection. WB-TG performed upon admission revealed a hypercoagulable profile vs. healthy controls. The patient was immediately initiated on corticosteroids with good response (i.e., hemoglobin values) and subsequent resolution of hemolysis within ten days. Antithrombotic prophylaxis was administered and no thrombotic events occurred. WB-TG may be a valuable tool in the clinical management of PNH, particularly for the early identification of hypercoagulability and tailoring anticoagulant regimens.</p>","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144532654","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}