Reduced cleavage of von willebrand factor by ADAMTS13 is associated with microangiopathic acute kidney injury following trauma
Acute kidney injury (AKI) is common after trauma, but contributory factors are incompletely understood. Increases in plasma von Willebrand Factor (vWF) with concurrent decreases in ADAMTS13 are associated with renal microvascular thrombosis in other disease states, but similar findings have not been shown in trauma. We hypothesized that molecular changes in circulating vWF and ADAMTS13 promote AKI following traumatic injury. VWF antigen, vWF multimer composition and ADAMTS13 levels were compared in plasma samples from 16 trauma patients with and without trauma-induced AKI, obtained from the Prehospital Air Medical Plasma (...
Source: Blood Coagulation and Fibrinolysis - January 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Readability and quality assessment of internet-based patient education materials related to deep vein thrombosis
Access to health-related internet information has increased dramatically during the past decade. A significant proportion of this information has been demonstrated to be set at too high a level for general comprehension. The goal of this study was to evaluate the readability and quality of information available on the internet relating to deep vein thrombosis (DVT). A search for ‘DVT’ and ‘Deep Vein Thrombosis’ was performed on five most commonly accessed search engines. Top hundred websites were reviewed. Website authorship was classified. Each site was assessed using the recognized quality and readability scoring...
Source: Blood Coagulation and Fibrinolysis - January 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Human coagulation factor IX: a systematic review of its characteristics
This article explained the coagulation process involved in FIX and the principle of haemophilia B caused by its absence. We searched and aligned the sequence information of IX in the NCBI's EST database to predict its structure and understand the molecular biological basis of its function. Further, by sorting out the development history of FIX, we summarized the general process of its isolation and purification. Finally, the clinical indications and complications of FIX were overviewed. Through a comprehensive description of the characteristics of FIX, we hope to give readers a clear understanding and put forward our views...
Source: Blood Coagulation and Fibrinolysis - January 1, 2022 Category: Hematology Tags: REVIEW ARTICLE Source Type: research

Evaluation of ISTH-BAT as a predictor for factor deficiency in haemophilia carriers: a single-centre experience
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: LETTER TO THE EDITOR Source Type: research

Multiple thrombotic sources and embolic events at arterial sites in a patient with protein-C deficiency
Protein-C deficiency causes a thrombophilic environment in circulation. Although venous thromboembolism is the most common presentation, arterial events are rare. A 45 year-old woman with protein-C deficiency presented with multiple thromboembolic events including bilateral renal infarct and inferior ST-elevation myocardial infarction 3-days after coronary angiography via femoral approach which were normal coronary arteries. Computed tomographic angiography and transesophageal echocardioraphy displayed multiple thrombi sources including left atrium, left subclavian artery, sinus valsalva and descending aorta. Unfractionate...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: CASE REPORTS Source Type: research

A refugee child with multiple patterned ecchymoses: a diagnostic dilemma between nonaccidental injury and primary hemostatic defect
A 4-year-old girl from Syria presented to the hospital with multiple bruises on her body. Bruises were observed in protected areas in a shape of fingerprints and objects, while no other bruises occurred during hospitalization. The parents also reported a history of bleeding diathesis from infancy. Both the initial laboratory evaluation and the secondary tests done for possible thrombocytopenia and coagulation factors deficiencies were normal. Thus, the nonaccidental injury protocol of the Hospital was activated, and the possibility of abuse was not quite evident. Investigation for platelet disorders followed. Platelet aggr...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: CASE REPORTS Source Type: research

Two heterozygous mutations associated with type I protein C deficiency in two Chinese independent families
To explore the pathogenesis of protein C (PC) deficiency in two independent families by mutations detection and bioinformatics analysis. The PC activity (PC:A) and PC antigen (PC:Ag) were detected by chromogenic substrate and ELISA, respectively. The PROC sequencing was performed to identify the mutational sites. The molecular pathogenesis of the mutations were studied by the conservation, bioinformatics and model analysis. The PC:A and PC:Ag of the proband 1 were observably reduced at 35 and 44%, respectively. Gene sequencing analysis revealed the p.Leu278Pro derived from a heterozygous c.833T>C point mutations in exon 9 ...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: MUTATION REPORT Source Type: research

Physician practices in evaluation and treatment of patients with generalized joint hypermobility and bleeding
The objectives of this study were to (1) evaluate hematologists’ diagnosis and management practices for patients with bleeding symptoms and generalized hypermobility spectrum disorder/hEDS and (2) determine future education and research priorities regarding bleeding symptoms within this population. Evaluate hematologists’ diagnosis and management practices for patients with bleeding symptoms and generalized hypermobility spectrum disorder/hEDS. Determine future education and research priorities regarding bleeding symptoms within this population. A web-based survey was sent to Hemostasis and Thrombosis Research Society ...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: SHORT COMMUNICATION Source Type: research

Naive haemophilia mice displayed different pattern of cytokine profiles of cytokine profiles changes might be associated with subclinical bleeding
Subclinical bleeding is a haemorrhage event not clinically detected in haemophilia, and no reliable method is available for predicting subclinical bleeding. We investigated whether haemophilia mice have subclinical haemorrhage and evaluated potential biomarkers including multiple cytokine changes to predict subclinical haemorrhage. Plasma from naïve FVIII−/− and FIX−/− mice and their wild-type counterparts (FVIII WT and FIX WT, respectively) were measured for prothrombin fragment 1 + 2 (F1 + 2) and multiple cytokines. Haemophilia mice with induced hemarthrosis were used as positive clinical bleeding contro...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Anticoagulation management post-transjugular intrahepatic portosystemic shunt in portal hypertension associated with myeloproliferative neoplasms
We describe failure of DOACs to prevent post-TIPS complications in two case reports of patients with MPN-associated pHTN and review the associated literature. We conducted a comprehensive search in Embase (embase.com), Scopus (scopus.org), and PubMed for existing data on MPN-associated pHTN post-TIPS procedure. Four studies (n = 251) of patients with pHTN post-TIPS were eligible (MPN, n = 143). A review of the literature suggests that patients with MPN-associated pHTN may be at higher risk for post-TIPS complications including stent thrombosis and stenosis, compared with other causes of thrombotic pHTN. DOAC use ha...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Significant difference between coagulation parameters and clinicopathological characteristics in breast cancer
The purpose of this research is to investigate the correlation between coagulation parameters and survival on patients with breast cancer. A total of 244 patients with breast cancer and 84 patients with metastatic advanced breast cancer admitted to the Second Affiliated Hospital of Chongqing Medical University from January 2017 to February 2019 were selected as the research objects. Their age, tumor size, status of estrogen receptor and progesterone receptor status, status of Her-2 receptor and lymph node were evaluated. Additional 99 cases of benign breast tumors were selected as the control group. All patients were newly...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

A multicentre real-world data study to assess the bleeding rate and management of patients with haemophilia A and factor VIII inhibitors in Argentina
The development of inhibitors against factor VIII (FVIII) concentrates is a severe complication of treatment for patients with haemophilia. We investigated annualized bleeding rates (ABRs) in patients in Argentina with haemophilia A with inhibitors and analysed potential differences between treatment strategies. This multicentre, retrospective, real-world data, cohort design study comprised ambulatory paediatric and adult patients with congenital haemophilia A and FVIII inhibitors treated according to standard clinical practice, with 12-months follow-up. Of 69 included patients, 39 (56.5%) received on-demand treatment, 13 ...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

In-vitro thromboelastographic characterization of reconstituted whole blood utilizing cryopreserved platelets
Conducting in-vitro thrombosis research presents numerous challenges, the primary of which is working with blood products, whether whole blood or fractionated whole blood, that have limited functional shelf-lives. As a result, being able to significantly prolong the clotting functionality of whole blood via fractionation and recombination promises greater accessibility via resource minimization in the realm of thrombosis research. Whole blood with CPDA1 from healthy volunteers was fractionated and stored as frozen platelet-free plasma (PFP, -20°C), refrigerated packed red blood cells (pRBCs, 4°C) and cryopreserved platel...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Relationship between serum soluble endothelial protein C receptor level and COVID-19 findings
This study has shown for the first time that serum sEPCR levels, which is a member of coagulation cascade and has also been reported to be associated with inflammation, is higher in patients with positive RT-qPCR test and patients with GGO or bilateral involvement on thorax CT regardless of the PCR result. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Thrombogenicity markers for early diagnosis and prognosis in COVID-19: a change from the current paradigm?
Standard biomarkers have been widely used for COVID-19 diagnosis and prognosis. We hypothesize that thrombogenicity metrics measured by thromboelastography will provide better diagnostic and prognostic utility versus standard biomarkers in COVID-19 positive patients. In this observational prospective study, we included 119 hospitalized COVID-19 positive patients and 15 COVID-19 negative patients. On admission, we measured standard biomarkers and thrombogenicity using a novel thromboelastography assay (TEG-6s). In-hospital all-cause death and thrombotic occurrences (thromboembolism, myocardial infarction and stroke) were re...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research