Characterization of zebrafish gp1ba mutant and modelling Bernard Soulier syndrome
The aim of this study is to model classical Bernard Soulier Syndrome in the zebrafish by targeting Gp1ba. We obtained gp1ba mutant embryos from Zebrafish International Resource Center and grew them to adulthood. The tail clips from these fish were used to prepare DNA and sequenced to identify heterozygotes. They were then bred to obtain homozygotes. The mutation was confirmed by DNA sequencing as a termination codon UAA in place of AAA codon at position 886 in the gp1ba transcript. Thus, at the Pro-295, the Gp1ba protein could be terminated. The blood from gp1ba homozygous and heterozygous mutants showed decreased ristocet...
Source: Blood Coagulation and Fibrinolysis - July 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Evaluation of coagulation parameters and impact of transfusion on coagulation in patients with beta thalassemia major
In this study, we evaluated whether or not the platelet function analyser (PFA-200) and thrombin generation test (TGT) would be useful tools to identify hypercoagulability and risk of thrombosis in thalassemia major patients.
The study included 50 patients with thalassemia major and 104 healthy control group. Pretransfusion and posttransfusion PFA-200 and TGT results were compared with control group. We found that median C/ADP and C/EPI values in the thalassemia major group were greater in both the pre and posttransfusion samples than the C/ADP and C/EPI results from the control group. The TGT results showed there was n...
Source: Blood Coagulation and Fibrinolysis - July 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Reversal of factor Xa inhibitors associated intracranial haemorrhage at a tertiary medical centre
This study is a singlecentre, retrospective chart review, including patients admitted between 1 January 2016 and 15 August 2019, who received 4F-PCC or andexanet for the management of FXaI-associated intracranial haemorrhage. Of the 45 patients included in this study, 23 patients were in the andexanet group and 22 were in the 4F-PCC group. At index admission, mean age was 76 years and the majority of patients (64%) were on apixaban with 33% presented with Glasgow Coma Scale 24 (GCS) score less than 12. At hospital discharge, 47% of patients in the andexanet group had died or discharged to hospice compared with 45% in the...
Source: Blood Coagulation and Fibrinolysis - July 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Epidemiological study about the mental state of patients after a pulmonary embolism or deep venous thrombosis event
The objective of this study was to investigate whether VTE alters the patients’ mental state. We gathered questionnaire data on 100 patients (59 men, 41 women; age ranging from 24 to 85 years) concerning psychological symptoms and body image. Our results show that after a VTE, patients develop psychological symptoms, some of which persist for more than 2 years. Examples of those symptoms include depression, intrusion, and increased scrutiny of the body. Intrusion (flashbacks, nightmares, and other traumatic sensations of reliving the thrombotic event) affected 69% of patients and may lead to social isolation, occasiona...
Source: Blood Coagulation and Fibrinolysis - July 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Thromboprophylaxis with argatroban in critically ill patients with sepsis: a review
During sepsis, an initial prothrombotic shift takes place, in which coagulatory acute-phase proteins are increased, while anticoagulatory factors and platelet count decrease. Further on, the fibrinolytic system becomes impaired, which contributes to disease severity. At a later stage in sepsis, coagulation factors may become depleted, and sepsis patients may shift into a hypo-coagulable state with an increased bleeding risk. During the pro-coagulatory shift, critically ill patients have an increased thrombosis risk that ranges from developing micro-thromboses that impair organ function to life-threatening thromboembolic ev...
Source: Blood Coagulation and Fibrinolysis - July 1, 2022 Category: Hematology Tags: REVIEW ARTICLE Source Type: research
Thrombosis-associated hypofibrinogenemia: novel abnormal fibrinogen variant FGG c.8G>A with oxidative posttranslational modifications
Here, we present the first case of fibrinogen variant FGG c.8G>A. We investigated the behaviour of this mutated fibrinogen in blood coagulation using fibrin polymerization, fibrinolysis, fibrinopeptides release measurement, mass spectrometry (MS), and scanning electron microscopy (SEM). The case was identified by routine coagulation testing of a 34-year-old man diagnosed with thrombosis. Initial genetic analysis revealed a heterozygous mutation in exon 1 of the FGG gene encoding gamma chain signal peptide. Fibrin polymerization by thrombin and reptilase showed the normal formation of the fibrin clot. However, maximal absor...
Source: Blood Coagulation and Fibrinolysis - June 1, 2022 Category: Hematology Tags: MUTATION REPORTS Source Type: research
Protein S Erlangen: a novel PROS1 gene mutation associated with quantitative protein S deficiency
The members of a Caucasian family were genetically analyzed on suspicion of hereditary protein S deficiency. A novel mutation, c.1904T>C, associated with severe quantitative protein S deficiency was found. The novel PROS1 mutation was identified by sequencing of the PROS1 gene coding sequence. The identified c.1904T>C point mutation results in p.Phe635Ser amino acid exchange, which is located in the Laminin G-like 2 domain of protein S. Computational analysis indicates that this amino acid exchange affects the correct folding of the protein S antigen. Furthermore, this mutation is located in a region of the Laminin G-like ...
Source: Blood Coagulation and Fibrinolysis - June 1, 2022 Category: Hematology Tags: MUTATION REPORTS Source Type: research
Coagulopathy following Crotaliπae snakebites in northeast Florida
Effects of Crotalinae envenomation vary by geographical areas and research into coagulopathy and effects of antivenom are needed to optimize management. This was a single-center retrospective review with testing on presentation and 4 h after; antivenom administration was noted and data analyzed overall and comparing envenomations. One hundred and nineteen snakebites evaluated with 59 identified as Crotalinae and half receiving antivenom. PT/aPTT was elevated in 20% of water moccasin/copperhead and 21% of rattlesnake bites. DIC-like syndrome occurred in 8% water moccasin/copperhead and 6% rattlesnake bites. Antivenom did ...
Source: Blood Coagulation and Fibrinolysis - June 1, 2022 Category: Hematology Tags: SHORT COMMUNICATION Source Type: research
In-hospital venous thromboembolism: are glucocorticoids a prime suspect?
The objective of the study was to determine whether glucocorticoid use is associated with an increased incidence of in-hospital VTE. We conducted a case–control study of patients with an in-hospital VTE from October 2015 to December 2019. Adult patient cases were identified by ICD-10 codes for acute venous thromboembolism. Controls were selected from all patients without a VTE diagnosis and matched by hospital length of stay and admission type (medical/surgical). Patients were excluded if they had a history of VTE, received therapeutic anticoagulation, or were pregnant. All patients were evaluated to determine the presen...
Source: Blood Coagulation and Fibrinolysis - June 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Evaluation of D-dimer levels measured by different analytical methods in COVID-19 patients
Clinicians experience some challenges due to the lack of standardization of test, although D-dimer is a prognostic marker for COVID-19. We compared the clinical and analytical performances of D-dimer results obtained from different devices, kits and methods in patients with a diagnosis of COVID-19. Thirty-nine patients with a diagnosis of COVID-19 and 24 healthy individuals were included in the study. D-dimer levels were measured with Innovance D-DIMER kit (immunoturbidimetric method) on Sysmex CS-2500 and BCS XP and VIDAS D-Dimer Exclusion II kit (enzyme-linked fluorescence method) on mini VIDAS. The studies of precision,...
Source: Blood Coagulation and Fibrinolysis - June 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Evaluation of the impact of changes in the autopsy rate on mortality trend of pulmonary embolism, Finland, 1996–2017
Pulmonary embolism is listed as a cause of death in fewer and fewer cases in the twenty-first century. Autopsies reveal undiagnosed pulmonary embolism at a significant rate, but fewer and fewer autopsies are being performed. It could be that deaths from pulmonary embolism are not decreasing, but are increasingly missed because of decreasing autopsy rate. Population-based registry data of all individuals with pulmonary embolism related death from the Finnish death certificate archive from 1996 to 2017 were collected. The pulmonary embolism mortality trend was analysed with linear regression and the association of pulmonary ...
Source: Blood Coagulation and Fibrinolysis - June 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Use of thrombolytic agents to treat neonatal thrombosis in clinical practice
Among children, neonates have the highest incidence of thrombosis. Thrombolytic agents are used for the management of life and/or organ-threatening thrombosis. Literature on the efficacy and safety of thrombolytic agents in neonates is limited. We reviewed the evidence on dosing, administration, monitoring and treatment duration of tissue plasminogen activator (tPA), streptokinase and urokinase (URK) in neonates (≤ 28days). A systematic literature search was conducted of current databases from inception until 31 March 2021. The initial search yielded 6881 articles and 18 were retained for review. tPA, streptokinase and U...
Source: Blood Coagulation and Fibrinolysis - June 1, 2022 Category: Hematology Tags: REVIEW ARTICLE Source Type: research
Age, sex and racial differences in fibrin formation and fibrinolysis within the healthy population
This study defines age, sex and racial differences of fibrin generation and fibrinolysis as measured by the OHP assay in a sample of healthy subjects. Further studies are warranted in diseased populations, where there is growing awareness of the role of global coagulation assay in defining prothrombotic and hypofibrinolytic states. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - February 11, 2022 Category: Hematology Tags: LETTER TO THE EDITOR Source Type: research
Refractory acquired haemophilia A in a patient with HIV treated with Emicizumab
Acquired haemophilia is a rare coagulopathy characterized by acquired inhibitors directed against clotting factors resulting in bleeding episodes. A middle-aged woman with HIV developed refractory haemophilia with bleeding episodes resulting in recurrent hospitalizations despite several rounds of bypassing agents and several lines of immunosuppressive agents. She was eventually successfully treated with Emicizumab and has not had any major bleeding episodes for 3 years since initiation of this treatment. Emicizumab, which is a bispecific, FVIII-mimetic therapeutic antibody, has considerably reduced the annualized bleedin...
Source: Blood Coagulation and Fibrinolysis - February 11, 2022 Category: Hematology Tags: CASE REPORTS Source Type: research
A challenging case of heparin-induced skin necrosis without thrombocytopenia
We present a case in which a patient treated for a pulmonary embolism with therapeutic unfractionated heparin (UFH) develops this adverse event. Symptoms were reversed with cessation of UFH and transition to bivalirudin without surgical debridement. The patient initially had anti-PF4 antibodies present but subsequent testing showed borderline antibodies and a negative serotonin release assay. After starting bivalirudin, the patient was later switched to fondaparinux without further consequences. This case illustrates the pathogenesis of HISN and provides an example of inconsistently abnormal laboratory values. Additionally...
Source: Blood Coagulation and Fibrinolysis - February 11, 2022 Category: Hematology Tags: CASE REPORTS Source Type: research
