Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis最新文献_第10页

Outcomes of recombinant activated factor VIIa (NovoSeven) therapy in glanzmann thrombasthenia: two case reports. 重组活化因子VIIa （NovoSeven）治疗格兰兹曼血栓缺乏症的疗效：2例报告。

IF 1.1

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis Pub Date : 2025-09-01 Epub Date: 2025-07-17 DOI: 10.1097/MBC.0000000000001380

Eman Almatar, Sondus Alsharidah, Omnia A Hashem

{"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":"10.1097/MBC.0000000000001380","url":null,"abstract":"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.","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":"293-295"},"PeriodicalIF":1.1,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12416894/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144651988","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}

引用次数: 0

The role of endothelial cell protein C receptor gene polymorphism in venous thromboembolic disease: a case report and literature review. 内皮细胞蛋白C受体基因多态性在静脉血栓栓塞性疾病中的作用：1例报告并文献复习。

IF 1.1

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis Pub Date : 2025-09-01 Epub Date: 2025-07-03 DOI: 10.1097/MBC.0000000000001373

Ilinca Savulescu-Fiedler, Roxana N Siliste, Corina Homentcovschi, Andrei F Gegiu, Dan Spataru, Luiza Dorobantu-Lungu, Madalina Selagea, Oana Stanca, Magdalena Bratu, Adriana L Gurghean

{"title":"The role of endothelial cell protein C receptor gene polymorphism in venous thromboembolic disease: a case report and literature review.","authors":"Ilinca Savulescu-Fiedler, Roxana N Siliste, Corina Homentcovschi, Andrei F Gegiu, Dan Spataru, Luiza Dorobantu-Lungu, Madalina Selagea, Oana Stanca, Magdalena Bratu, Adriana L Gurghean","doi":"10.1097/MBC.0000000000001373","DOIUrl":"10.1097/MBC.0000000000001373","url":null,"abstract":"Deep vein thrombosis (DVT) is one of the most serious cardiovascular disease in all age groups and implies an elevated risk for death, via pulmonary thromboembolism. Classically described as Virchow's triad, the main three factors implied are venous stasis, vascular wall lesion, and an increased coagulation vs. fibrinolytic mechanism. These pathophysiological conditions are fulfilled when various predisposing factors meet the environmental risk factors. The clinical judgment points that if, in certain situations, when the likelihood of an inherited propensity for thrombosis is very high, the search for a specific trait has to be done, and sometimes beyond the current guideline recommendations, because it could be related to future recurrences and could identify potential other relatives at increased risk for this serious pathology. In this article, we discuss a case involving a 45-year-old man who was admitted for sudden-onset dyspnea. The clinical presentation, along with a family history of thrombophilia, raised initial suspicion of pulmonary embolism, which was confirmed by a chest computed tomography (CT) scan with contrast enhancement. Both the patient and his family members underwent extensive tests for hypercoagulable disorders, which returned positive results. We review the current evidence and reassess the importance of thrombophilia testing in similar patients, highlighting the role of EPCR in venous thromboembolic disease.","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":"242-252"},"PeriodicalIF":1.1,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144610918","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}

引用次数: 0

Novel prognostic factor in thrombotic thrombocytopenic purpura: human leukocyte antigen class II. 血栓性血小板减少性紫癜的新预后因素：人白细胞抗原II类。

IF 1.1

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis Pub Date : 2025-09-01 Epub Date: 2025-08-08 DOI: 10.1097/MBC.0000000000001372

Cevat İlteriş Kikili, Demet Kivanç, Damla Ortaboz, Hayriye Şentürk Çiftçi, Murat Özbalak, Mustafa Nuri Yenerel, Meliha Nalçaci, Muhlis Cem Ar, Fatma Savran Oğuz, Sevgi Kalayoğlu Beşişik

引用次数: 0

Silent progression of hemophilic arthropathy of the elbow: case reports of three pediatric patients. 肘部血友病的无声进展：3例儿科患者的病例报告。

IF 1.1

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis Pub Date : 2025-09-01 Epub Date: 2025-07-17 DOI: 10.1097/MBC.0000000000001382

Atsuki Yamashita, Mika Mori, Yotaro Umezawa, Tomoko Ashikaga, Chiai Nagae, Ryota Koyama, Takeshi Arai, Naoki Haraguchi, Yuki Saito, Atsuko Fujikawa, Masashi Taki

{"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":"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.","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":"288-292"},"PeriodicalIF":1.1,"publicationDate":"2025-09-01","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}

引用次数: 0

Management of gastrointestinal bleeding in a patient with type 3 von Willebrand disease and inhibitors to von Willebrand factor: a case report. 3型血管性血友病和血管性血友病因子抑制剂患者胃肠道出血的治疗：1例报告

IF 1.1

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis Pub Date : 2025-09-01 Epub Date: 2025-06-20 DOI: 10.1097/MBC.0000000000001371

Ena Ranković, Marijo Vodanović, Marino Narančić, Dražen Pulanić, Josipa Belev, Mirjana Kalauz, Desiree Coen Herak, Marko Rajić, Ana Boban

{"title":"Management of gastrointestinal bleeding in a patient with type 3 von Willebrand disease and inhibitors to von Willebrand factor: a case report.","authors":"Ena Ranković, Marijo Vodanović, Marino Narančić, Dražen Pulanić, Josipa Belev, Mirjana Kalauz, Desiree Coen Herak, Marko Rajić, Ana Boban","doi":"10.1097/MBC.0000000000001371","DOIUrl":"10.1097/MBC.0000000000001371","url":null,"abstract":"von Willebrand disease (VWD) is a rare bleeding disorder caused by deficiency of von Willebrand factor (VWF). Development of inhibiting alloantibodies to VWF in VWD after exposure to factor concentrates is very rare. We present a 56-year-old male patient with type 3 VWD and history of inhibitors to VWF who was hospitalized due to severe bleeding from the rectal angiodysplasia, which happened 6 months after radical irradiation therapy of the prostate adenocarcinoma. He was treated locally with argon plasma coagulation and application of peptide hydrogel, which resulted in progression of lesions to rectal ulcer covering two-thirds of the rectal mucosa. In addition to almost daily transfusions of red blood cells, he received recombinant activated factor VIIa (rFVIIa), tranexamic acid, FXIII concentrate and activated prothrombin complex concentrate (APCC). Infusion of VWF/FVIII concentrate resulted in reappearance of FVIII inhibitors. Ultimately, the bleeding stopped after introduction of off-label emicizumab, platelet transfusions and local application of the platelet-rich fibrin (PRF).","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":"279-282"},"PeriodicalIF":1.1,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144478523","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}

引用次数: 0

The prevalence and associated factors of aspirin resistance in JAK2 V617F-mutated essential thrombocythemia and polycythemia vera. jak2v617f突变的原发性血小板增多症和真性红细胞增多症患者阿司匹林抵抗的患病率及相关因素

IF 1.1

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis Pub Date : 2025-09-01 Epub Date: 2025-06-27 DOI: 10.1097/MBC.0000000000001376

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

{"title":"The prevalence and associated factors of aspirin resistance in JAK2 V617F-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":"10.1097/MBC.0000000000001376","url":null,"abstract":"The primary aim was to determine the prevalence of aspirin resistance in JAK2 V617F-mutated essential thrombocythemia and polycythemia vera patients. Secondary objectives included analyzing risk factors for aspirin resistance including JAK2 V617F allele burden. This cross-sectional study included patients aged at least 18 years with JAK2 V617F-mutated essential thrombocythemia and polycythemia vera who were prescribed aspirin. Blood samples were taken to measure JAK2 V617F allele burden and serum thromboxane B2 (TXB2). Aspirin resistance was defined by serum TXB2 levels greater than 570 pg/ml. Clinical characteristics, laboratory data, JAK2 V617F 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). JAK2 V617F allele burden was not significantly correlated with high serum TXB2 ( r2 = 0.087, P = 0.45). The prevalence of aspirin resistance was 100% in JAK2 V617F-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.","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":"261-267"},"PeriodicalIF":1.1,"publicationDate":"2025-09-01","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}

引用次数: 0

Whole blood thrombin generation hypercoagulable profile in a patient with hemolytic crisis due to paroxysmal nocturnal hemoglobinuria: a case report. 阵发性夜间血红蛋白尿致溶血性危象患者的全血凝血酶生成高凝特征：1例报告。

IF 1.1

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis Pub Date : 2025-09-01 Epub Date: 2025-06-27 DOI: 10.1097/MBC.0000000000001377

Angela Napolitano, Luca Spiezia, Cristiana Bulato, Elena Campello, Paolo Simioni

{"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":"10.1097/MBC.0000000000001377","url":null,"abstract":"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.","PeriodicalId":520571,"journal":{"name":"Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis","volume":" ","pages":"283-287"},"PeriodicalIF":1.1,"publicationDate":"2025-09-01","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}

引用次数: 0

An alternative method to assess the long-term stability of frozen plasmas for coagulation testing. 一种评估凝固试验冷冻血浆长期稳定性的替代方法。

IF 1.1

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis Pub Date : 2025-09-01 Epub Date: 2025-08-08 DOI: 10.1097/MBC.0000000000001374

Pascale Laroche, Joffrey Feriel, Odile Chachlica, Geneviève Tanala Marolahy, François Depasse

引用次数: 0

Current epidemiology of deaths for disseminated intravascular coagulation. 播散性血管内凝血死亡的当前流行病学。

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis Pub Date : 2025-07-01 Epub Date: 2025-07-04 DOI: 10.1097/MBC.0000000000001369

Camilla Mattiuzzi, Giuseppe Lippi

引用次数: 0

Hereditary hepatic fibrinogen storage disease with a novel fibrinogen variant FGG c.1113T>A ( fibrinogen Seoul III ). 遗传性肝纤维蛋白原储存病伴新型纤维蛋白原变异FGG c.1113T> a（纤维蛋白原Seoul III）。

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis Pub Date : 2025-07-01 Epub Date: 2025-06-12 DOI: 10.1097/MBC.0000000000001358

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

引用次数: 0