Blood Coagulation & Fibrinolysis最新文献_第8页

Thrombopoietin receptor agonist and rituximab combination therapy in patients with refractory primary immune thrombocytopenia. 针对难治性原发性免疫血小板减少症患者的血小板生成素受体激动剂和利妥昔单抗联合疗法。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-02-14 DOI: 10.1097/MBC.0000000000001283

Michał Witkowski, Wiktoria Ryżewska, Tadeusz Robak

{"title":"Thrombopoietin receptor agonist and rituximab combination therapy in patients with refractory primary immune thrombocytopenia.","authors":"Michał Witkowski, Wiktoria Ryżewska, Tadeusz Robak","doi":"10.1097/MBC.0000000000001283","DOIUrl":"10.1097/MBC.0000000000001283","url":null,"abstract":"The aim of our study was to evaluate the efficacy of this therapy in patients with refractory primary immune thrombocytopenia. It is crucial to develop alternative treatment methods for this patient group in order to achieve better response. This combination therapy combines two different mechanisms of action, which is promising in terms of targeting pathophysiology of immune thrombocytopenia. We conducted a retrospective study, which included all patients who were diagnosed with refractory primary immune thrombocytopenia and received TPO-RA and rituximab at the General Hematology Department, Copernicus Memorial Hospital in Lodz, Poland. We assessed the response, time to response and treatment-free remission (TFR). After 1 month of treatment, the complete response (CR1, PLT >100 g/l) was achieved in 62.5% patients, and response (R1, PLT >30 g/l) was achieved in 62.5% patients. The median PLT was 175 × 10 9 /l. Within 1 month of treatment, 87.5% of patients achieved TFR. Adequately, after 6 months, CR6 and R6 was 62.5 and 75%. The median PLT was 182 × 10 9 /l. Treatment-free remission 6 months after completion was in 50% of patients. The study group achieved response to treatment, which suggests that combination of TPO-RA and rituximab is effective and relatively well tolerated. Prospective study on larger group of patients is needed to better evaluate the efficiency and safety of this treatment.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"108-114"},"PeriodicalIF":1.1,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139740312","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The link between high factor VIII to protein C ratio values and poor liver function after major hepatectomy. 高因子 VIII 与蛋白 C 比值与大肝切除术后肝功能不佳之间的联系。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-01-30 DOI: 10.1097/MBC.0000000000001277

Patricia Duque, Jose María Perez-Peña, Lleimi Alarcon-Perez, Luis Olmedilla, Jesús Alberto Varela, Cristina Pascual, Ana María Rodriguez-Huerta, José Manuel Asencio, Jose Ángel Lopez-Baena, Ignacio Garutti

{"title":"The link between high factor VIII to protein C ratio values and poor liver function after major hepatectomy.","authors":"Patricia Duque, Jose María Perez-Peña, Lleimi Alarcon-Perez, Luis Olmedilla, Jesús Alberto Varela, Cristina Pascual, Ana María Rodriguez-Huerta, José Manuel Asencio, Jose Ángel Lopez-Baena, Ignacio Garutti","doi":"10.1097/MBC.0000000000001277","DOIUrl":"10.1097/MBC.0000000000001277","url":null,"abstract":"Our goal was to assess the coagulation profile in the immediate postoperative time after major liver surgery and its association with the liver function. Our hypothesis is that a decreased synthesis of the coagulation factor levels reflects an impaired liver synthesis following hepatic resection and will be associated with poor outcomes. This is a prospective, observational study recruiting consecutive patients scheduled for major liver resection in a tertiary hospital. Coagulation profile was assessed by conventional assays, viscoelastic assays and coagulation factor levels preoperatively and, on postoperative days 1, 2 and 6. Factor VIII to protein C (FVIII/PC) ratio has been used as a surrogate marker of hemostatic imbalance. Liver function was measured with conventional and indocyanine green (ICG) clearance tests, which were obtained preoperatively and on postoperative days 1 and 2. Sixty patients were recruited and 51 were included in the study. There is a clear increase in FVIII/PC ratio after surgery, which was significantly associated with low liver function, being more pronounced beyond postoperative day 2 and in patients with poorer liver function ( P < 0.001). High FVIII/PC ratio values were significantly associated with higher postoperative morbidity, prolonged ICU and hospital stay and less survival ( P < 0.05). High FVIII/PC ratio on postoperative day 2 was found to be predictor of posthepatectomy liver failure (PHLF; area under the ROC curve = 0.8129). Early postoperative high FVIII/PC ratio values are associated with low liver function, PHLF and poorer outcomes in patients undergoing major hepatic resection.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"82-93"},"PeriodicalIF":1.1,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139671248","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Falsely prolonged prothrombin time test in a patient with erythrocytosis: a case report. 一名红细胞增多症患者的凝血酶原时间检测错误延长：病例报告。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-01-25 DOI: 10.1097/MBC.0000000000001276

Meysam Aghajani Daronkola, Ali Dabbagh, Mahmood Shams, Shadi Tabibian, Soudabeh Hosseini, Seyed Mehrab Safdari, Akbar Dorgalaleh

{"title":"Falsely prolonged prothrombin time test in a patient with erythrocytosis: a case report.","authors":"Meysam Aghajani Daronkola, Ali Dabbagh, Mahmood Shams, Shadi Tabibian, Soudabeh Hosseini, Seyed Mehrab Safdari, Akbar Dorgalaleh","doi":"10.1097/MBC.0000000000001276","DOIUrl":"10.1097/MBC.0000000000001276","url":null,"abstract":"The prothrombin time (PT) test is commonly used to monitor deficiencies in coagulation factors. A prolonged PT may indicate a deficiency of factors II, V, VII, X, and fibrinogen, or the presence of an inhibitor. However, further tests are required to differentiate between a true factor deficiency and the presence of an inhibitor. It is important to note that falsely prolonged PT can lead to misdiagnosis and inappropriate clinical intervention that can have life-threatening consequences. A 19-year-old woman with elevated hematocrit levels and prolonged PT was diagnosed with secondary erythrocytosis due to cyanotic congenital heart disease with ventricular septal defect (VSD). However, further investigation revealed that the prolonged PT result was false. Excess citrate in the blood sample, caused by polycythemia, led to this misleading outcome, resulting in unnecessary and potentially harmful treatment. This incident emphasizes the importance of laboratory personnel and clinicians being aware of the test's limitations. Not only should specialists in thrombosis and hemostasis possess this knowledge, but it is also pertinent for general laboratory staff, as well as laboratory directors and specialists. The significance of accurate laboratory testing for the proper diagnosis and treatment of patients is highlighted in this case.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"136-138"},"PeriodicalIF":1.1,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139671246","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Bleeding disorders in Saudi Arabia, causes and prevalence: a review. 沙特阿拉伯的出血性疾病、病因和发病率：综述。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-02-15 DOI: 10.1097/MBC.0000000000001286

Tareg M Belali

{"title":"Bleeding disorders in Saudi Arabia, causes and prevalence: a review.","authors":"Tareg M Belali","doi":"10.1097/MBC.0000000000001286","DOIUrl":"10.1097/MBC.0000000000001286","url":null,"abstract":"As bleeding disorders are a worldwide health concern, Saudi Arabia is experiencing a notable prevalence of such disorders. Studying the frequency and cause of hemostatic disorders is the key to successful clinical interventions and instigating effective public policies that limit the spread of such disorders. The current review aims to highlight the major findings of the body of literature that has investigated the causes, prevalence, and major challenges associated with bleeding disorders in the country. The current review summarizes the major findings of different studies that have been conducted in Saudi Arabia regarding different bleeding disorders. Multiple causes and symptoms of bleeding disorders have been reported by different studies. Some studies investigated the genetic aspect of bleeding disorders and revealed specific mutations in coagulation factor genes influencing the symptoms of different bleeding disorders. Moreover, rare bleeding disorders such as Glanzmann thrombasthenia and Henoch-Schönlein purpura, have been reported in different regions of Saudi Arabia. Combining clinical presentations, genetic factors, and epidemiological data, the current review of the literature provides a comprehensive insight into bleeding disorders in the kingdom. This will help in advancing the diagnostic capabilities and genetic counseling enhancing management strategies and therapeutic interventions benefiting bleeding disorder patients and the kingdom.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"67-72"},"PeriodicalIF":1.1,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10990020/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139740305","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Severe thrombocytopenia associated to bevacizumab in a patient with scleroderma, gastrointestinal angiodysplasias and refractory gastrointestinal bleeding. 一名患有硬皮病、胃肠道血管增生症和难治性胃肠道出血的患者因使用贝伐珠单抗而导致严重血小板减少。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-02-14 DOI: 10.1097/MBC.0000000000001284

Eugenia Perez Lloveras, Juan Manuel Michelangelo, Carlos Gustavo Videla, Maria Laura Gonzalez, Veronica Privitera, Marcelo Martin Serra, Carolina Vazquez

{"title":"Severe thrombocytopenia associated to bevacizumab in a patient with scleroderma, gastrointestinal angiodysplasias and refractory gastrointestinal bleeding.","authors":"Eugenia Perez Lloveras, Juan Manuel Michelangelo, Carlos Gustavo Videla, Maria Laura Gonzalez, Veronica Privitera, Marcelo Martin Serra, Carolina Vazquez","doi":"10.1097/MBC.0000000000001284","DOIUrl":"10.1097/MBC.0000000000001284","url":null,"abstract":"This case report discusses the medical history of a 64-year-old woman diagnosed with scleroderma and diffuse gastrointestinal angiodysplasia. The patient received bevacizumab (BVZ) therapy to address gastrointestinal bleeding that was unresponsive to endoscopic treatment. Subsequently, she developed severe thrombocytopenia. Although there were suspicions of an immune-mediated mechanism resulting from BVZ treatment, the laboratory results did not provide conclusive evidence. The patient underwent transfusions, received gamma globulin, and was treated with Romiplostim. Over time, her platelet levels gradually improved, and the bleeding was successfully controlled. It's worth noting that BVZ-induced thrombocytopenia is a relatively rare yet severe adverse effect. Recognizing and understanding the mechanisms behind thrombocytopenia is essential for developing safer treatment approaches. Further research is required to identify potential risk factors associated with this condition.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"141-146"},"PeriodicalIF":1.1,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139740310","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Real-world comparative effectiveness of dalteparin and enoxaparin for venous thromboembolism prophylaxis. 达肝素和依诺肝素预防静脉血栓栓塞的实际效果比较。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-02-15 DOI: 10.1097/MBC.0000000000001281

Anna L Di Mauro, Lewis J Austin, Jasmine Zande, Karl Winckel, Rodney Neale, Keshia R De Guzman

{"title":"Real-world comparative effectiveness of dalteparin and enoxaparin for venous thromboembolism prophylaxis.","authors":"Anna L Di Mauro, Lewis J Austin, Jasmine Zande, Karl Winckel, Rodney Neale, Keshia R De Guzman","doi":"10.1097/MBC.0000000000001281","DOIUrl":"10.1097/MBC.0000000000001281","url":null,"abstract":"Venous thromboembolism (VTE) is a preventable cause of significant morbidity and mortality in hospitalized patients world-wide. In Australia, the low-molecular weight heparins (LMWHs) enoxaparin or dalteparin are usually used as first-line prophylaxis for VTE, though there is uncertainty whether dalteparin has the same effectiveness as enoxaparin in real-world settings. This is relevant because dalteparin is less renally cleared and may be more cost effective than enoxaparin. The aim of this study was to explore VTE event incidence in a general cohort of hospitalized adult inpatients who were prescribed enoxaparin or dalteparin for VTE prophylaxis. A retrospective observational study was conducted at a quaternary hospital in Brisbane, Australia, of patients who had experienced a hospital-acquired VTE from 1 September 2021 to 1 March 2023. Patients were identified from routinely collected data following an in-hospital VTE event, and further data was retrieved retrospectively from the integrated electronic Medical Record (ieMR). Incidence and type of VTE events, LMWH-prescribing patterns, and risk factors were assessed. The incidence of VTE events were similar across the dalteparin and enoxaparin cohorts (42.1 events/10 000 patients vs. 34.4 events/10 000 patients, respectively), although patients prescribed enoxaparin had a higher number of risk factors, particularly obesity and active cancer. Our research indicates comparable incidence of VTE in patients prescribed dalteparin compared with enoxaparin in an Australian hospital general cohort of adult inpatients. Dalteparin may be as effective as enoxaparin for VTE prophylaxis in a real-world cohort of patients, and as such dalteparin may be considered a suitable alternative to enoxaparin for VTE prophylaxis. Further research including large randomized controlled trials are required to confirm these results.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"101-107"},"PeriodicalIF":1.1,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139740309","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Hereditary factor X deficiency in America survey: impact on quality of life and burden of disease in patients and caregivers. 美国遗传性 X 因子缺乏症调查：对患者和护理人员生活质量和疾病负担的影响。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-02-21 DOI: 10.1097/MBC.0000000000001275

Brian Branchford, Kim Clark, Richard H Stanford, Denise A Garner, Shirley P Huang, Eric Wolford

{"title":"Hereditary factor X deficiency in America survey: impact on quality of life and burden of disease in patients and caregivers.","authors":"Brian Branchford, Kim Clark, Richard H Stanford, Denise A Garner, Shirley P Huang, Eric Wolford","doi":"10.1097/MBC.0000000000001275","DOIUrl":"10.1097/MBC.0000000000001275","url":null,"abstract":"Hereditary factor X deficiency (HFXD) is a rare bleeding disorder causing delayed haemostasis and potentially life-threatening bleeds. Patient/caregiver burden and diagnosis path have not been well characterized.The aim of this study was to: describe the diagnosis path, disease burden, and HFXD impact on quality of life (QoL) in patients and caregivers.This was a prospective, cross-sectional, web-based survey of patients with HFXD and caregivers addressing the patient/caregiver experience, QoL, humanistic and unmet needs.Thirty patients and 38 caregivers completed the survey with mean ages 24.7 and 44.6 years, respectively. Mean age at diagnosis was 4.1 years. The diagnostic process was somewhat/very difficult for 23% of patients and 26% of caregivers. Approximately half (53%) received single factor replacement (SFR) as prophylaxis or on-demand. Most patients (71%) reported regular prophylaxis treatment. Over one-fourth (27%) reported treatment with fresh frozen plasma. Bleeding episodes were less common in patients using SFR versus non-SFR: three bleeds or fewer were reported by 92% SFR and 75% non-SFR patients. HFXD patients reported low well being in work/school/social activities with mean HFXD-adapted Hemophilia Well being Index. Patient symptoms negatively impacted caregiver burden with a mean HFXD-adapted Hemophilia Caregiver Index (±SD) of 15.9 (4.6), but also unexpectedly had a positive impact on self-worth and inner strength.To our knowledge, this is the first study to assess patient and caregiver burden of HFXD and impact on QoL. Improvements in symptom recognition, prompt diagnosis, and adherence to expert recommendations for treatment could improve QoL and decrease burden on HFXD patients and caregivers.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"73-81"},"PeriodicalIF":1.1,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10990012/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139671247","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Lupus anticoagulant hypoprothrombinemia syndrome with multiple and high-titer antiphospholipid antibodies strongly interfered with coagulation assays. 狼疮抗凝物低凝血酶原血症综合征伴有多种高滴度抗磷脂抗体，对凝血测定产生强烈干扰。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-04-05 DOI: 10.1097/MBC.0000000000001282

Motoki Sugasaki, Shingen Nakamura, Keisuke Teramoto, Minami Urushihara, Yusuke Inoue, Takayuki Nakao, Yasuhiko Nishioka, Masataka Sata

引用次数: 0

Mixing studies for lupus anticoagulant: does it matter how we mix? 狼疮抗凝剂的混合研究：如何混合重要吗？

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-02-14 DOI: 10.1097/MBC.0000000000001288

Vanja Radišić Biljak, Matea Tomas, Ivana Lapić

引用次数: 0

Sustained good response to rituximab in acquired von Willebrand syndrome. 获得性冯-威廉综合征患者对利妥昔单抗的持续良好反应。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-04-01 Epub Date: 2024-04-05 DOI: 10.1097/MBC.0000000000001278

Jayna Mistry, Gillian Clare Lowe, Will Lester, Charles Llewellyn Percy

引用次数: 0