Blood Coagulation & Fibrinolysis最新文献

Is the combination of two automated rapid assays for diagnosis of heparin-induced thrombocytopenia necessary? 肝素诱发血小板减少症的诊断是否需要结合两种自动快速检测？

IF 1.2 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-12-01 Epub Date: 2024-10-15 DOI: 10.1097/MBC.0000000000001327

Erika Jani, Margherita Bozzola, Elmar Marco Zagler, Massimo Daves

{"title":"Is the combination of two automated rapid assays for diagnosis of heparin-induced thrombocytopenia necessary?","authors":"Erika Jani, Margherita Bozzola, Elmar Marco Zagler, Massimo Daves","doi":"10.1097/MBC.0000000000001327","DOIUrl":"10.1097/MBC.0000000000001327","url":null,"abstract":"Heparin-induced thrombocytopenia (HIT) is an immune-mediated condition characterized by a decrease in platelet count and an increased thrombotic risk. HIT event is caused by antiplatelet factor/heparin (PF4/H) antibodies that can activate the platelets. The diagnosis of HIT is based on a clinical evaluation and laboratory results. Aim of our study was to evaluate whether the combined use of two rapid assays for diagnosis of HIT provides a diagnostic advantage over the use of a single automate assay. We extracted from the laboratory informatic system all the determinations requested for the detection of antibodies against heparin/PF4 complexes from July 2020 to June 2024 (n. 229). In our laboratory, total antibodies against heparin/PF4 complex [HemosIL HIT-Ab-(PF4-H), Instrumentation Laboratory] and IgG Ab (HemosIL AcuStar HIT-IgG, Instrumentation Laboratory) are measured simultaneously with two different instruments. Two hundred six samples tested negative for both methods, 23 samples tested positive for at least one method and nine samples tested positive for both methods. The grade of concordance between the two assays shows a weighted Kappa of 0.536 (moderate agreement). No sample tested positive only for IgG-Ab. The sensitivity of HIT-Ab-(PF4-H) was 1, whereas the specificity was 0.95. For the HIT-IgG (PF4-H) method, sensibility and specificity were 0.77 and 1, respectively. Our results suggest that performing these two tests simultaneously does not provide additional useful information in patients with suspicion of HIT. The total Ab assay seems to be sufficient, as it shows higher sensitivity although at the expense of lower specificity.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142494645","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

Variation among venous thromboembolism risk assessment tools for postcesarean patients: a retrospective cohort study. 针对剖宫产后患者的静脉血栓栓塞风险评估工具之间的差异：一项回顾性队列研究。

IF 1.2 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-12-01 Epub Date: 2024-10-14 DOI: 10.1097/MBC.0000000000001325

Alshaima Fraoug Eltayeb Ahmed, Seeba Zachariah, Amal Hassan Ismail, Caitlin M Gibson

{"title":"Variation among venous thromboembolism risk assessment tools for postcesarean patients: a retrospective cohort study.","authors":"Alshaima Fraoug Eltayeb Ahmed, Seeba Zachariah, Amal Hassan Ismail, Caitlin M Gibson","doi":"10.1097/MBC.0000000000001325","DOIUrl":"10.1097/MBC.0000000000001325","url":null,"abstract":"Venous thromboembolism (VTE) risk in pregnant women is four to five-fold higher than in nonpregnant women, and the risk of VTE is an additional four-fold higher after Cesarean section compared to normal vaginal delivery. Recommendations regarding anticoagulant prophylaxis are inconsistent across international guidelines, and VTE remains one of the leading causes of maternal morbidity and mortality. This study aimed to compare the need for postcesarean anticoagulation for VTE prophylaxis based on three major guidelines and our own institutional protocol. It was a retrospective cohort study that reviewed the medical records of patients who underwent a cesarean section at a tertiary-level care hospital in the United Arab Emirates (UAE). The need for anticoagulation was assessed using clinical tools from the American College of Obstetricians and Gynecologists (ACOG), Royal College Obstetricians and Gynecologists (RCOG), American College of Chest Physicians (ACCP), and the study site hospital protocol. A total of 1134 postcesarean women, aged 18-55 years, were included in the study. Most patients (87%) were at moderate risk for VTE. According to the study site hospital tool, 90.7% qualified for anticoagulant prophylaxis, while the ACOG, RCOG, and ACCP tools indicated that 0.5, 90.9, and 36.7% qualified, respectively. Enoxaparin was the primary anticoagulant used in 95% of cases. Only one patient developed VTE during the follow-up period. Anticoagulation needs assessment tools vary extensively in their estimations, highlighting the need for a uniform tool across multiple societies to establish a consistent standard of care and guide the development of evidence-based, site-specific protocols.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142457303","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

Challenges in managing severe homozygous protein c deficiency: a case report. 管理严重同型蛋白 c 缺乏症的挑战：一份病例报告。

IF 1.2 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-11-05 DOI: 10.1097/MBC.0000000000001332

Eman Almatter, Sondus Alsharidah, Mona Bourhama, Maha Bourusly, Mohamad Adel Obaid

引用次数: 0

A novel SERPINC1 c.119G>A (p.Cys40Tyr) mutation with variable clinical expression in an Indian family. 一个印度家庭中出现的临床表现各异的新型 SERPINC1 c.119G>A (p.Cys40Tyr) 突变。

IF 1.2 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-11-04 DOI: 10.1097/MBC.0000000000001333

Kranti Patil, Asha Shah, Gurpreet Saini, Shreyas Tawde, Shruti Kharat, Fiza Jivani, Aniket Kamble, Shrimati Shetty

引用次数: 0

A novel γ-chain mutation p.Asp318His in a Chinese family with dysfibrinogenemia. 一个中国纤维蛋白原不良血症家族的新型γ-链突变 p.Asp318His

IF 1.2 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-10-25 DOI: 10.1097/MBC.0000000000001329

Haiyue Zhang, Weifeng Shen

{"title":"A novel γ-chain mutation p.Asp318His in a Chinese family with dysfibrinogenemia.","authors":"Haiyue Zhang, Weifeng Shen","doi":"10.1097/MBC.0000000000001329","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001329","url":null,"abstract":"Background: Congenital dysfibrinogenemia is characterized by reduced fibrinogen activity, but normal immunoreactive fibrinogen levels. Here, we present a novel case with an elevated risk of thrombosis.Methods: Coagulation assays, gene analysis, in silico tools, sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE), fibrin polymerization, thrombin generation assay, and electron microscopy scanning were utilized to elucidate the pathogenic mechanism.Results: The proband manifested with a normal immunologic fibrinogen (2.13 g/l) but reduced functional fibrinogen (0.39 g/l). Subsequent genetic analysis unveiled a novel heterozygous mutation, c.1030G>C (p.Asp318His), in the γ-chain D domain of fibrinogen, which was highly conserved in homologous species and led to enhanced thrombin generation capability. The ability of the proband's fibrinogen to polymerize was significantly impaired, with decreased final turbidity. Scanning electron microscopy indicated that the fibers of the proband were thinner than normal, with smaller pores. Thromboelastography (TEG) results demonstrated prolonged K time, decreased angle value, and a normal confidence interval value in the proband.Conclusion: We present a novel case displaying the γAsp318His mutation, which resulted in dysfibrinogenemia.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2024-10-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142603019","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 role of factor XIII in patient blood management. XIII 因子在患者血液管理中的作用。

IF 1.2 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-09-26 DOI: 10.1097/MBC.0000000000001326

Miodrag Žunić, Nino Vreča, Sebastjan Bevc

引用次数: 0

Severe acquired Factor VII deficiency complicating an aplastic anemia, successfully treated with corticosteroids. 严重的获得性因子 VII 缺乏症并发再生障碍性贫血，使用皮质类固醇治疗成功。

IF 1.2 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-09-23 DOI: 10.1097/MBC.0000000000001323

Batton Romain, Chamouni Pierre, Carrette Marion, Sauvêtre Gaëtan, Barbay Virginie

引用次数: 0

Improved prevention of bleeding episodes with emicizumab in 3 patients with concomitant hemophilia A and von Willebrand disease. 埃米珠单抗能有效预防 3 名同时患有血友病 A 和冯-威廉氏病的患者的出血发作。

IF 1.2 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-09-05 DOI: 10.1097/MBC.0000000000001324

Kristin T Ansteatt, Jonathan C Roberts, Jackie M Helms, Michael D Tarantino

{"title":"Improved prevention of bleeding episodes with emicizumab in 3 patients with concomitant hemophilia A and von Willebrand disease.","authors":"Kristin T Ansteatt, Jonathan C Roberts, Jackie M Helms, Michael D Tarantino","doi":"10.1097/MBC.0000000000001324","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001324","url":null,"abstract":"The typical phenotype of hemophilia A (HA) is that of frequent bleeding episodes, up to several per month, unless prophylactic factor VIII (FVIII) replacement or alternatives are given. Related bleeding may be heightened in severity or frequency by co-morbid bleeding disorders. Based on the reported prevalence of von Willebrand disease (VWD) of up to 1% of the general population, the co-existence of HA and VWD occurs, but is likely less recognized. Prophylactic FVIII replacement may or may not adequately prevent bleeding in persons with HA and mild VWD, and plasma-derived concentrates containing FVIII and von Willebrand factor (VWF) may be used for more successful bleeding prophylaxis. However, therapy adherence remains problematic for many reasons, one being treatment via intravenous access. Emicizumab is a nonfactor subcutaneous prophylactic therapy for HA that may overcome this concern. We describe three patients, with severe HA and VWD, for whom regular FVIII/VWF prophylaxis was deemed inadequate. FVIII/VWF prophylaxis was replaced with weekly prophylactic injections of the bispecific monoclonal antibody, emicizumab. When the patients were transitioned to emicizumab, all experienced a significant reduction in their annualized bleed rate (ABR). Although the mechanism of action does not directly affect or replace VWF function, emicizumab may be an effective prophylaxis alternative to FVIII/VWF concentrate in patients with concomitant severe HA and VWD.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142153117","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

Cancer-associated thrombosis: the role of inherited thrombophilia. 癌症相关血栓形成：遗传性血栓性疾病的作用。

IF 1.2 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-09-01 Epub Date: 2024-07-26 DOI: 10.1097/MBC.0000000000001317

Anita Zia, Mahmood Shams, Ali Dabbagh, Milad Shahsavari, Akbar Dorgalaleh

引用次数: 0

Identification of HLA alleles involved in immune thrombotic thrombocytopenic purpura patients from Turkey. 土耳其免疫性血栓性血小板减少性紫癜患者的 HLA 等位基因鉴定。

IF 1.2 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-09-01 Epub Date: 2024-07-24 DOI: 10.1097/MBC.0000000000001318

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

{"title":"Identification of HLA alleles involved in immune thrombotic thrombocytopenic purpura patients from Turkey.","authors":"Cevat İlteriş Kikili, Demet Kivanç, Damla Ortaboz, Hayriye Şentürk Çiftçi, Mustafa Murat Özbalak, Mustafa Nuri Yenerel, Meliha Nalçaci, Muhlis Cem Ar, Fatma Savran Oğuz, Sevgi Kalayoğlu Beşişik","doi":"10.1097/MBC.0000000000001318","DOIUrl":"10.1097/MBC.0000000000001318","url":null,"abstract":"Thrombotic thrombocytopenic purpura (TTP) is one of the rare group disorders classified as thrombotic microangiopathies (TMAs). Approximately 90% of TTP developed immune-mediation by the formation of antibodies against the enzyme ADAMTS-13. The exact cause is unknown. To establish an association between human leukocyte antigen (HLA) and autoimmune basis, as susceptibility or protection against the disease, we contributed a study aiming to evaluate the role of HLA in immune-mediated TTP (iTTP). Considering epidemiological factors such as age, sex, ethnicity, and geographical origins, we contributed the study in our country, Turkey, which consist of a very heterogeneous population. Patients' data collection was retrospectively from electronic database on two University hospitals having big therapeutic apheresis service. Control arm was healthy people registered as stem cell donors matched in terms of age and sex. The frequency of HLA-DRB1 and HLA-DQB1 alleles between acquired TTP and the control group was compared using the chi-square method. Yates correction and logistic regression were performed on these results. A total of 75 iTTP patients and 150 healthy individuals enrolled to the study. HLA-DRB1∗11, HLA-DQB1∗03, HLA-DRB1∗11:01, HLA-DRB1∗14:01, HLA-DRB1∗13:05, HLA-DRB1∗11 + HLA-DQB1∗03 allele pair and HLA-DRB1∗15 + HLA- DQB1∗06 were proved to be susceptibility allele pairs for iTTP. HLA-DRB1∗15, HLA-DRB1∗01:01, HLA-DRB1∗07:01, HLA-DRB1∗13:01, HLA-DRB1∗14:54, HLA-DQB1∗05:01, HLA-DQB1∗02:02 and HLA-DRB1∗07 + HLA-DQB1∗02 allele pair were found to be protective against iTTP. Our findings support an association with iTTP across very heterogenous populations in Turkey.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141854671","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