Blood Coagulation & Fibrinolysis最新文献_第9页

Perioperative hemostasis management in patients with von Willebrand disease: an institutional experience. 冯-威廉氏病患者围手术期止血管理：机构经验。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-03-01 Epub Date: 2023-12-20 DOI: 10.1097/MBC.0000000000001273

Rosa Toenges, Wolfgang Miesbach, Kaja Ludwig, Beate Krammer-Steiner

{"title":"Perioperative hemostasis management in patients with von Willebrand disease: an institutional experience.","authors":"Rosa Toenges, Wolfgang Miesbach, Kaja Ludwig, Beate Krammer-Steiner","doi":"10.1097/MBC.0000000000001273","DOIUrl":"10.1097/MBC.0000000000001273","url":null,"abstract":"Objectives: Patients with von Willebrand disease (vWD) undergoing surgery are routinely treated with von Willebrand factor (vWF)/factor VIII (FVIII) concentrate to control bleeding risk, but consensus is lacking on optimal dosing. This study aimed to evaluate the efficacy and safety of tailored doses of vWF/FVIII concentrate according to intervention-associated bleeding risk in vWD patients undergoing surgery.Methods: This was a retrospective analysis of vWD patients who underwent surgical procedures at a haemophilia centre. Patients received vWF/FVIII concentrate with dosage and duration of treatment dependent on intervention type (dental, gynaecological, abdominal or orthopaedic/traumatic) and bleeding risk (moderate/high).Results: Eighty-three surgical procedures (42 patients) were included. Median preoperative loading doses of vWF/FVIII concentrate were 29.9 IU/kg and 35.7 IU/kg for interventions with moderate ( n = 16) or high ( n = 67) bleeding risk, respectively. The median perioperative dose was highest in orthopaedic or trauma-related surgery (140 IU/kg) and lowest in dental or gynaecological interventions (76.4 IU/kg and 80.0 IU/kg, respectively). During follow-up, no bleeding or other complications were observed in 95% of patients.Conclusions: Individually tailored doses of vWF/FVIII concentrate according to intervention-associated bleeding risk were effective in preventing postoperative bleeding, with few complications observed. These doses may be used as guidance in routine clinical care.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"49-55"},"PeriodicalIF":1.1,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139097152","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

Double jeopardy, glomangiopericytoma and Glanzmann thrombasthenia resulting in recurrent epistaxis: a case report. 双重危险、格兰氏血管瘤和格兰兹曼血栓形成症导致复发性鼻衄：病例报告。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-03-01 Epub Date: 2023-12-20 DOI: 10.1097/MBC.0000000000001272

Emna Hammami, Léa Fath, Christian Debry, Dominique Desprez

{"title":"Double jeopardy, glomangiopericytoma and Glanzmann thrombasthenia resulting in recurrent epistaxis: a case report.","authors":"Emna Hammami, Léa Fath, Christian Debry, Dominique Desprez","doi":"10.1097/MBC.0000000000001272","DOIUrl":"10.1097/MBC.0000000000001272","url":null,"abstract":"Glanzmann thrombasthenia is a rare bleeding disorder induced by inherited defects of the platelet membrane αIIbβ3 glycoprotein. Glomangiopericytoma, on the other hand, is a very rare sinonasal tumor demonstrating a perivascular myoid phenotype. We herein report the first described case in the literature of Glanzmann thrombasthenia and glomangiopericytoma. The patient is a 40-year-old man diagnosed with type 1 Glanzmann thrombasthenia who presented with repetitive and profuse posterior epistaxis initially managed with platelet transfusions and recombinant activated factor VII (rFVIIa). Due to the unresolved epistaxis, nasal endoscopy was performed revealing a vascularized tumor. Subsequently, a sphenopalatine artery embolization followed by a surgical excision of the tumor was performed. The pathology report diagnosis of the tumor was glomangiopericytoma. This case sheds the lights on a very rare cause of epistaxis in a patient with Glanzmann thrombasthenia, with a challenging multidisciplinary management. A local cause of epistaxis should always be considered even in case of a diagnosed bleeding disorder, especially when the bleeding is recurrent.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"62-65"},"PeriodicalIF":1.1,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139097239","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

Diagnostic value of clot formation parameters determined by rotational thromboelastometry in 63 patients with congenital dysfibrinogenemia. 通过旋转血栓弹性测定法确定的 63 名先天性纤维蛋白原血症患者血凝块形成参数的诊断价值。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-03-01 Epub Date: 2024-01-19 DOI: 10.1097/MBC.0000000000001274

Tomas Simurda, Rita Marchi, Alessandro Casini, Marguerite Neerman-Arbez, Miroslava Drotarova, Ingrid Skornova, Jana Zolkova, Zuzana Kolkova, Dusan Loderer, Monika Brunclikova, Kristina Maria Belakova, Jan Stasko

{"title":"Diagnostic value of clot formation parameters determined by rotational thromboelastometry in 63 patients with congenital dysfibrinogenemia.","authors":"Tomas Simurda, Rita Marchi, Alessandro Casini, Marguerite Neerman-Arbez, Miroslava Drotarova, Ingrid Skornova, Jana Zolkova, Zuzana Kolkova, Dusan Loderer, Monika Brunclikova, Kristina Maria Belakova, Jan Stasko","doi":"10.1097/MBC.0000000000001274","DOIUrl":"10.1097/MBC.0000000000001274","url":null,"abstract":"Rotational thromboelastometry (ROTEM) is a global hemostasis assay. The diagnosis added value of ROTEM in congenital dysfibrinogenemia remains to be established. The aim of this study was to analyze clot formation by ROTEM in a cohort of dysfibrinogenemic patients and to establish correlations with genotype, clinical features, and coagulation parameters. The study included genetically confirmed congenital dysfibrinogenemia cases (n = 63) and healthy controls ( n = 50). EXTEM, INTEM, FIBTEM tests were used to measure ROTEM parameters, that is, clotting time (CT), clot formation time (CFT), maximal clot firmness (MCF) and amplitude 10 min after CT (A10). The ISTH bleeding assessment tool was used to determine bleeding episodes. CT (INTEM) was statistically significantly shorter in congenital dysfibrinogenemia patients compared to controls while CFT (EXTEM) was prolonged. Patients's MCF in EXTEM, INTEM, and FIBTEM were similar to controls while A10 (FIBTEM) was statistically significantly lower. Fibrinogen activity was positively correlated with fibrinogen antigen, A10 and MCF in all three assays. Bleeding phenotypes were observed in 23 (36.5%) patients. Only CFT in EXTEM and CT in INTEM were statistically different in patients with bleeding phenotype versus controls. Carriers of the FGA mutation p.Arg35His had a CT (EXTEM) slightly prolonged and a reduced A10 (FIBTEM) compared to controls. Some ROTEM parameters were able to distinguish congenital dysfibrinogenemia patients from controls, and patients with a bleeding phenotype. Prolonged CFT in EXTEM were associated with congenital dysfibrinogenemia and bleeding phenotype. Bleeding episodes in most patients were generally mild and prevalence of thrombosis was very low.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"56-61"},"PeriodicalIF":1.1,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10927300/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139511465","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

Exploring antithrombin: insights into its physiological features, clinical implications and analytical techniques. 探索抗凝血酶：深入了解其生理特点、临床意义和分析技术。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-03-01 Epub Date: 2023-12-15 DOI: 10.1097/MBC.0000000000001271

Muhammad Saboor, Hassan A Hamali, Abdullah A Mobarki, Aymen M Madkhali, Gasim Dboie

{"title":"Exploring antithrombin: insights into its physiological features, clinical implications and analytical techniques.","authors":"Muhammad Saboor, Hassan A Hamali, Abdullah A Mobarki, Aymen M Madkhali, Gasim Dboie","doi":"10.1097/MBC.0000000000001271","DOIUrl":"10.1097/MBC.0000000000001271","url":null,"abstract":"Antithrombin is an essential protein that acts as a natural anticoagulant in the human body. It is synthesized by the liver and belongs to the serine protease inhibitors, which are commonly referred to as the SERPINS superfamily. The antithrombin molecule comprises 432 amino acids and has a molecular weight of approximately 58 200 D. It consists of three domains, including an amino-terminal domain, a carbohydrate-rich domain, and a carboxyl-terminal domain. The amino-terminal domain binds with heparin, whereas the carboxyl-terminal domain binds with serine protease. Antithrombin is a crucial natural anticoagulant that contributes approximately 60-80% of plasma anticoagulant activities in the human body. Moreover, antithrombin has anti-inflammatory effects that can be divided into coagulation-dependent and coagulation-independent effects. Furthermore, it exhibits antitumor activity and possesses a broad range of antiviral properties. Inherited type I antithrombin deficiency is a quantitative disorder that is characterized by low antithrombin activity due to low plasma levels. On the other hand, inherited type II antithrombin deficiency is a qualitative disorder that is characterized by defects in the antithrombin molecule. Acquired antithrombin deficiencies are more common than hereditary deficiencies and are associated with various clinical conditions due to reduced synthesis, increased loss, or enhanced consumption. The purpose of this review was to provide an update on the structure, functions, clinical implications, and methods of detection of antithrombin.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"43-48"},"PeriodicalIF":1.1,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139097240","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

Interpreting coagulation mixing study results in the era of direct oral anticoagulants. 解释直接口服抗凝剂时代的混凝研究结果。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-01-01 Epub Date: 2023-11-06 DOI: 10.1097/MBC.0000000000001267

Moon Joo Kim, Eric Salazar, Bonnie Philips, Lawrence Rice, Brian Castillo, Christopher Leveque, Jian Chen

{"title":"Interpreting coagulation mixing study results in the era of direct oral anticoagulants.","authors":"Moon Joo Kim, Eric Salazar, Bonnie Philips, Lawrence Rice, Brian Castillo, Christopher Leveque, Jian Chen","doi":"10.1097/MBC.0000000000001267","DOIUrl":"10.1097/MBC.0000000000001267","url":null,"abstract":"Interpretation of coagulation mixing studies is complicated by interference arising from direct oral anticoagulants (DOACs), which are increasingly prescribed. In this retrospective study, we reviewed 1035 consecutive coagulation mixing studies performed from 2017 to 2021. Three hundred and ninety-nine cases with normal prothrombin time (PT) and activated partial thromboplastin time (aPTT) were excluded. aPTT mixing studies were performed at time 0 and after 60 min of incubation. We confirmed the presence of interfering factors with additional laboratory testing, medication records, and medical history. Mixing corrected most prolonged PT samples (93%), but 32 cases showed incomplete correction. Of these 32 cases, 18 were confounded by DOAC use, and 3 by factor V (FV) inhibitor. We observed an unusual pattern of prolongation of aPTT after incubation, which was previously considered a characteristic of specific factor inhibitors, most commonly FVIII inhibitor. However, we found that lupus anticoagulant (28%) and DOAC (25%) contributed to this pattern similarly as specific factor inhibitors (28%). Coagulation laboratories should be aware of interference arising from DOACs and other factors in PT/aPTT mixing studies, especially in some unusual correction patterns.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"23-26"},"PeriodicalIF":1.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138294521","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

Clot waveform analysis in acute promyelocytic leukemia. 急性早幼粒细胞白血病的血块波形分析。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-01-01 Epub Date: 2023-11-08 DOI: 10.1097/MBC.0000000000001265

Malek Terras, Wijden El Borgi, Wafa Betbout, Rabeb Jaied, Fatma Ben Lakhal, Sarra Fekih Salem, Ons Ghali, Emna Gouider

{"title":"Clot waveform analysis in acute promyelocytic leukemia.","authors":"Malek Terras, Wijden El Borgi, Wafa Betbout, Rabeb Jaied, Fatma Ben Lakhal, Sarra Fekih Salem, Ons Ghali, Emna Gouider","doi":"10.1097/MBC.0000000000001265","DOIUrl":"10.1097/MBC.0000000000001265","url":null,"abstract":"The aim of this study was to evaluate the activated partial thromboplastin time (APTT) and prothrombin time (PT)-based clot waveform analysis (CWA) in patients diagnosed with acute promyelocytic leukemia (APL). APTT-based and PT-based CWA parameters of patients diagnosed with APL were analyzed and compared with healthy volunteers. Four APTT-CWA parameters were noted, maximum velocity corresponding to the first peak of the first derivative (max1), maximum acceleration corresponding to the first peak of the second derivative (max2) and the corresponding peak times of max1 and max2 (Tmax1, Tmax2). For the PT-CWA, two PT-CWA parameters were noted, maximum velocity (max1') and the corresponding timing (Tmax1'). The results were expressed in medians. Mann-Whitney U test was used to compare the CWA parameters. Correlations were examined using the Spearman correlation test. Tmax1 and Tmax2 were significantly prolonged in patients with APL in comparison with healthy volunteers. Although max1 and max2 were lower in APL patients compared with healthy volunteers, no significant difference was noted. There was a strong and significant correlation between the DIC score and the parameters max1, max2 and max1' and a very strong and significant correlation between fibrinogen levels and max1, max2 and max1'. When comparing DIC patients with hypofibrinogenemia and DIC without hypofibrinogenemia, a significant difference was noted in max1, max2, Tmax1 and Tmax2. The APTT and PT-based CWA analysis is a good tool to evaluate the bleeding tendency in APL, as it offers a novel approach for evaluating global hemostasis, predicting the bleeding risk and delivering improvements to APL patients management.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"27-31"},"PeriodicalIF":1.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138294520","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

Utility of the international society on thrombosis and hemostasis-bleeding assessment tool in the diagnosis of patients who suspected of platelet function disorders. 利用国际上关于血栓形成和止血出血的评估工具在诊断疑似血小板功能障碍患者中的应用。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-01-01 Epub Date: 2023-11-02 DOI: 10.1097/MBC.0000000000001264

Massoumeh Shahbazi, Minoo Ahmadinejad, Amir Teimourpour

{"title":"Utility of the international society on thrombosis and hemostasis-bleeding assessment tool in the diagnosis of patients who suspected of platelet function disorders.","authors":"Massoumeh Shahbazi, Minoo Ahmadinejad, Amir Teimourpour","doi":"10.1097/MBC.0000000000001264","DOIUrl":"10.1097/MBC.0000000000001264","url":null,"abstract":"The ISTH-BAT is a structured bleeding assessment tool to record and help diagnose patients with possible bleeding disorders. However, a few studies evaluated the utility of ISTH-BAT in diagnosing patients with platelet function defects (PFDs). In this study, we evaluated the diagnostic utility of ISTH-BAT in predicting PFDs among patients suspected of PFDs. Forty patients suspected of PFDs and 21 normal healthy controls were evaluated by the ISTH-BAT scoring system, light transmission aggregometry (LTA), ATP-releasing assays (lumi-aggregometry), and expression of CD62P for diagnosis of PFDs. Among 40 patients suspected of PFDs, 10 were diagnosed as PFDs using lumiaggregometry and CD62P. The ISTH-BAT score in patients suspected of PFDs [(6, interquartile range (IQR) 1-8] and patients with PFDs was significantly higher than the control group (0; IQR 0-0) ( P < 0.001). Receiver operating characteristic curves indicate that ISTH-BAT is not able to discriminate patients with PFDs from those without PFDs (areas under the curve of 0.620 (95% confidence interval 0.415-0.825). The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of the ISTH-BAT in predicting the presence of PFDs, respectively, were 40, 73.3, 33.3, and 78.6% in the cut-off ISTH-BAT at least 4 in adult men, at least 6 in adult women, and at least 3 in children (age < 18). The ISTH-BAT scoring system has good discriminatory power in diagnosing patients with PFDs from healthy controls but is ineffective in differentiating them from those without PFDs.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"8-13"},"PeriodicalIF":1.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138294523","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

Commercial human frozen plasmas for local, cross-site and long-term comparability of coagulation analysers. 用于凝血分析仪的本地、异地和长期可比性的商用人体冷冻血浆。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-01-01 Epub Date: 2023-11-25 DOI: 10.1097/MBC.0000000000001268

Amélie Launois, Sara Zia Chahabi, Floriane Devaux, Isabelle Amouroux, Claire Flaujac

{"title":"Commercial human frozen plasmas for local, cross-site and long-term comparability of coagulation analysers.","authors":"Amélie Launois, Sara Zia Chahabi, Floriane Devaux, Isabelle Amouroux, Claire Flaujac","doi":"10.1097/MBC.0000000000001268","DOIUrl":"10.1097/MBC.0000000000001268","url":null,"abstract":"Laboratory compliance implies to correlate instruments for coagulation parameter with a wide range of measure using patient samples or commercialized sets of frozen plasmas. The aim of this study was to evaluate the intra, inter-reproducibility and long-term stability of ExpertCor Routine (ECR) plasma sets (Stago) on different parameters. The study was realized in two laboratories on four different instruments. Inter-site and intra-site correlation of ECR sets for PT, aPTT, Fibrinogen, INR, factor V (FV) UFH and LMWH anti-Xa and intra-reproducibility of DDimer (DDI), factor VIII (FVIII:C) and antithrombin (AT) assays were tested. To evaluate ECR long-term stability, samples were tested until 180 after delivery in one laboratory. Intra-site evaluation correlation coefficients is around 1. All predefined criteria to fulfil good comparability between inter-site instruments are met with Passing slopes between 0.9 and 1.1 and intercepts ranging from -0.62 to 2.83%. Long-term stability evaluation does not show any deviation over 180 days for aPTT, fibrinogen, DDI, UFH, LMWH but a drift for FV with STA-NeoPTimal reagent. On contrary, AT and FVIII:C are not stable. PT in second has an excellent stability unlike PT in percentage. Our study validates the use of ECR sets for correlation between instruments and inter-sites agreement, as for parameters claimed on the products than for factor V and FVIII:C. The evaluation of stability confirming the possible extension of use for 180 days after delivery except for FVIII:C and AT. These plasmas sets are an excellent alternative to local plasma patient use to perform instrument comparison.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"14-22"},"PeriodicalIF":1.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138486618","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 coagulation factor XI deficiency: a rare or neglected disease? Results from a retrospective, single-centre cohort in northern Italy. 遗传性凝血因子 XI 缺乏症：罕见还是被忽视的疾病？意大利北部一个单一中心队列的回顾性研究结果。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-01-01 Epub Date: 2023-11-29 DOI: 10.1097/MBC.0000000000001270

Simone Canovi, Maria Cristina Leone, Luca Depietri, Maria Rosaria Veropalumbo, Annalisa Pilia, Maria Granito, Antonio Bonanno, Annamaria Casali, Rossana Colla, Angelo Ghirarduzzi

{"title":"Hereditary coagulation factor XI deficiency: a rare or neglected disease? Results from a retrospective, single-centre cohort in northern Italy.","authors":"Simone Canovi, Maria Cristina Leone, Luca Depietri, Maria Rosaria Veropalumbo, Annalisa Pilia, Maria Granito, Antonio Bonanno, Annamaria Casali, Rossana Colla, Angelo Ghirarduzzi","doi":"10.1097/MBC.0000000000001270","DOIUrl":"10.1097/MBC.0000000000001270","url":null,"abstract":"To examine real-life clinical data regarding hereditary factor XI (FXI) deficiency from a secondary care centre. Retrospective review of clinical records for every FXI:C 0.7 IU/ml or less reported from 2012 to 2020. Seventy-nine patients were included. Six (7.6%) had a severe deficiency (FXI:C <0.2 IU/ml). Only 55 (69.6%) patients were referred to the Haemostasis Centre. Among them, six (15%) were subsequently not identified at increased haemorrhagic risk before a surgical/obstetrical procedure. Thirty-three (41.8%) experienced at least one bleeding event, minor (25 patients) and/or major (16 patients). Minor bleedings were predominantly spontaneous and more frequent in women, major events were mainly provoked. No correlation was found between FXI:C and risk of bleeding ( P = 0.9153). Lower FXI:C, but not a positive bleeding history, was related with higher likelihood of being referred to the Haemostasis Centre ( P = 0.0333). Hereditary FXI deficiency prevalence is likely underestimated, real-life clinical practices outside reference centres could be suboptimal.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"32-36"},"PeriodicalIF":1.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138486628","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

Increased contact activated endogenous thrombin potential in pregnant women with preeclampsia. 子痫前期孕妇接触活化的内源性凝血酶潜能增加。

IF 1.1 4区医学

Blood Coagulation & Fibrinolysis Pub Date : 2024-01-01 Epub Date: 2023-11-30 DOI: 10.1097/MBC.0000000000001269

Anne Cathrine Godtfredsen, Yaseelan Palarasah, Britta Blume Dolleris, Jan Stener Jørgensen, Johannes Jakobsen Sidelmann, Jørgen Brodersen Gram

{"title":"Increased contact activated endogenous thrombin potential in pregnant women with preeclampsia.","authors":"Anne Cathrine Godtfredsen, Yaseelan Palarasah, Britta Blume Dolleris, Jan Stener Jørgensen, Johannes Jakobsen Sidelmann, Jørgen Brodersen Gram","doi":"10.1097/MBC.0000000000001269","DOIUrl":"10.1097/MBC.0000000000001269","url":null,"abstract":"Preeclampsia is a worldwide contributor to maternal and fetal morbidity and mortality. Women with preeclampsia are in a hyper-coagulable state with increased risk of thromboembolic disease later in life compared with normal pregnant women. The contact system (CAS) in plasma can mediate thrombin generation and is an important contributor to thrombus growth, but the activation of CAS during pregnancy complicated by preeclampsia is not yet elucidated, and CAS may play a role in the pathophysiology of preeclampsia. Therefore, the aim of the study is to address thrombin generation, and in particular, the capacity of the CAS-mediated pathway in patients with preeclampsia compared with pregnant controls. One hundred and seventeen women with preeclampsia and matched controls were included. The project was registered at www.clinicaltrials.gov as NCT04825145. CAS and tissue factor induced thrombin generation, proteins C and S, antithrombin, and histidine-rich glycoprotein (HRG) were assessed. Women with preeclampsia had significantly increased CAS and tissue factor-induced endogenous thrombin potential (ETP), and HRG compared with controls, P = 0.022, P = 0.024, and P = 0.02, respectively. The concentrations of protein C and antithrombin were significantly reduced in the preeclampsia group, P = 0.024 and P < 0.0001, respectively. No significant difference in the concentration of protein S was detected, P = 0.06. This study demonstrates a significant increased CAS-induced ETP and an overall decrease of important regulators of coagulation in women with preeclampsia compared with controls. These aspects can contribute to the hyper-coagulable state characterizing preeclampsia.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":" ","pages":"1-7"},"PeriodicalIF":1.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10836780/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138486629","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