Direct oral anticoagulant neutralization by activated charcoal DOAC-Remove for thrombophilia screening
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: LETTER TO THE EDITOR Source Type: research
A homozygous loss-of-function mutation in GP1BB causing variable clinical phenotypes in a family with Bernard–Soulier syndrome
Bernard–Soulier syndrome is a rare autosomal recessive bleeding disorder and has a low incidence. Bernard–Soulier syndrome is caused by the deficiency of glycoprotein GPIb-V-IX complex, a receptor for von Willebrand factor and is characterized by thrombocytopenia, giant platelets and bleeding tendency. We are reporting three members of a same family with variable phenotypic clinical presentation. The index case is a 20-year-old boy who has a frequent presentation with epistaxis, and low platelet counts (25 × 109/l). He had been hospitalized multiple times and received platelet transfusions. His brother and cousin ...
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: CASE REPORTS Source Type: research
Weekly low-dose recombinant factor VIIa prophylaxis in Glanzmann thrombasthenia
Glanzmann thrombasthenia is an inherited disease causing bleeding episodes due to platelet dysfunction. The standard treatment for moderate-severe bleeding is platelet transfusion. Recombinant factor VIIa (rFVIIa) is successfully used in bleeding episodes and invasive procedures. Here, we present a patient with Glanzmann thrombasthenia, whose bleeding episodes could only be controlled by rFVIIa. The patient is a 28 years old male, who has had frequent bleeding episodes unresponsive to local hemostatic agents and tranexamic acid and had an anaphylactoid reaction to platelet transfusion. We started the patient on a low-dose ...
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: CASE REPORTS Source Type: research
Novel splicing (c.6529-1G>T) and missense (c.1667G>A) mutations causing factor V deficiency
In this study, we investigated the genetic basis in an African American patient with factor V activity 3%. Custom sequence capture and targeted next-generation (NGS) sequencing of the F5 gene were undertaken followed by PCR and Sanger sequencing. Two novel variants were identified. In silico analyses correlated clinically with the patient's factor V activity and hemorrhagic tendency. A review of the literature regarding these genomic alterations is presented. We described two novel mutations causing moderate FVD. The first, Chr1:g.169483698C>A with cDNA change (F5):c.6529-1G>T, occurred in a conserved nucleotide at the can...
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: MUTATION REPORTS Source Type: research
Identification of two novel mutations in three children with congenital factor VII deficiency
In this study, we aimed to identify genetic defects and analyze their relationships with phenotype in three Chinese FVIID patients. The diagnosis of FVIID was made based on FVII coagulant activity (FVII:C) levels assessed through prothrombin time assay. Direct sequencing and protein modeling were performed to detect genetic mutations and the resulting protein expression. Patient 1, a 2-year-old girl, presented with mild bleeding and was found to have a FVII:C of 0.2% and a compound heterozygous F7 Cys389Gly/Cys115Arg mutation. Patient 2, a 7-year-old boy, consulted for moderate bleeding and was found to have a FVII:C of 0....
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: MUTATION REPORTS Source Type: research
Clinical factors associated with adverse outcomes in the acute period of management of submassive pulmonary embolism
The objective of current analysis was to determine predictors associated with adverse outcomes in submassive pulmonary embolism patients. This was a single-center, retrospective chart review identifying patient characteristics and clinical factors associated with adverse outcomes within the management of patients presenting to the emergency department for submassive pulmonary embolism. A total of 122 admissions for submassive pulmonary embolism were included. Among these patients, 41% (n = 50) of admissions had an adverse outcomes. Fluid volume was associated with adverse events in an incremental manner (odds ratio 2.1...
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: SHORT COMMUNICATION Source Type: research
Evaluation of 4Ts score inter-rater agreement in patients undergoing evaluation for heparin-induced thrombocytopenia
The American Society of Hematology and American College of Chest Physicians heparin-induced thrombocytopenia guidelines recommend calculation of a pretest probability score prior to performing laboratory testing, and the 4Ts score is commonly used. Inter-rater agreement of the 4Ts score has been evaluated, but limited data are available regarding the reliability of the 4Ts score when performed by nonexpert clinicians. The purpose of this study was to Compare 4Ts scores calculated by medical teams to an expert. A single-center observational study was conducted in patients evaluated for heparin-induced thrombocytopenia over ...
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Identification of a novel mutation in congenital afibrinogenemia in Iranian patients
Congenital afibrinogenemia is a rare autosomal recessive disorder that is caused by defects in the fibrinogen. Fibrinogen is a hexameric glycoprotein made of two pairs of three homologous polypeptide chains including Aα, Bβ, and γ that are encoded by three genes named FGA, FGB, and FGG. We aim to study four Iranian families who were referred to our lab for molecular diagnosis of afibrinogenemia. Genomic DNA was extracted from whole blood and Sanger sequencing was performed using primers for all exons and exon-intron junctions of FGA, FGB, and FGG genes. Pathogenicity of the variants was predicted using different in-sili...
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Expression and methylation status of vascular endothelial growth factor and thrombospondin-1 genes in congenital factor XIII-deficient patients with intracranial hemorrhage
This study was conducted on 40 participants with FXIII, 20 of whom experienced ICH (cases), and 20 who did not (controls). Methylation pattern, gene expression, and plasma protein level were assessed using bisulfite sequencing PCR, quantitative real-time PCR, and ELISA. We found a partially methylated pattern for both VEGF and TSP-1 (P > 0.05). VEGF mRNA levels of the case group were significantly higher than those of the control group (P 0.05). Plasma VEGF and TSP-1 concentrations in the case group were higher, but not statistically significant (P > 0.05). Our findings showed no obvious correlation between VE...
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
No correlation between mean platelet volume and carotid artery thickness in patients with diabetes at intermediate or high risk for cardiovascular diseases
The number of patients with diabetes with a risk of cardiovascular diseases (CVDs) is increasing worldwide, leading to a higher demand for evaluating atherosclerosis. Recently, the mean platelet volume (MPV) available from complete blood count is gaining attention as a marker of underlying atherosclerotic lesions. In the current study, we examined whether MPV can predict carotid atherosclerosis in patients with diabetes at an intermediate or high risk for CVD. A total of 224 patients with diabetes aged 36–85 years who underwent carotid ultrasound examination were assessed. The risk of CVD was evaluated using the Suita sc...
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Effect of unfractionated heparin and low molecular weight heparin on the clotting of platelet-reduced whole blood: an in-vitro study utilizing thromboelastography
Treatment of venous thromboembolism with concomitant thrombocytopenia is challenging. The platelet threshold for safe administration of anticoagulants is under debate, with minimum platelet count of 50 × 109/l being recommended as the safe cutoff. However, some evidence suggests administration of anticoagulants may still be safe at platelet levels of 30 × 109/l. Therefore, we developed an in-vitro thromboelastography (TEG) study to examine the effect of therapeutic or prophylactic levels of unfractionated heparin (UFH) and low molecular weight heparin (LMWH) on the clotting profile of platelet-reduced whole blood...
Source: Blood Coagulation and Fibrinolysis - July 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
A little heparin goes a long way: effect of therapeutic levels of unfractionated heparin on the heparin-induced thrombocytopenia-platelet factor 4 ELISA antibody assay
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - May 20, 2021 Category: Hematology Tags: LETTER TO THE EDITOR Source Type: research
Increased resistance to fibrinolysis activation in patients with coronavirus disease 2019: a case series
We describe seven critically ill and seven noncritically ill patients with coronavirus disease 2019 infection. Two viscoelastic assays were performed with ClotPro technology, activated by extrinsic system test and recombinant tissue plasminogen activator challenge test. Coagulation profile presents a marked hypercoagulability with increased resistance to fibrinolysis, reflected by tissue plasminogen activator test. Our pathological observations show that the hypercoagulative status described in these patients is, at least partially, secondary to fibrinolysis shutdown. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - May 20, 2021 Category: Hematology Tags: CASE REPORTS Source Type: research
Acquired factor V inhibitor in the setting of coronavirus disease 2019 infection
We present a case of an acquired factor V inhibitor likely caused by coronavirus disease 2019 infection. Bleeding was manifested by severe anemia requiring frequent red-cell transfusion, left psoas muscle hematoma, and left retroperitoneal cavity hematoma. Factor V activity was less than 1% and the factor V inhibitor titer was 31.6 Bethesda units. Severe acute respiratory syndrome coronavirus 2 RNA testing of the nasopharynx was positive 2 weeks before presentation and continued to be positive for 30 days. The patient failed treatment with intravenous immunoglobulin and dexamethasone. Three cycles of plasmapheresis with fr...
Source: Blood Coagulation and Fibrinolysis - May 20, 2021 Category: Hematology Tags: CASE REPORTS Source Type: research
Vascular activation is a strong predictor of mortality in coronavirus disease 2019 patients on the ICU
Respiratory failure in coronavirus disease 2019 (COVID-19) patients is one of the most frequent causes for referral to the ICU. A significant percentage of these patients does not survive the infection due to thromboembolic complications. Furthermore, the vascular system seems also to be involved in the pathogenesis. To investigate the role of hemostasis and endothelium on the outcome of COVID-19 patients admitted to the ICU. Blood was drawn from 16 ICU COVID-19 patients for hemostatic analysis. Patients were followed-up till discharge (n = 11) or death (n = 5). Parameters related to both coagulation and fibrinolys...
Source: Blood Coagulation and Fibrinolysis - May 20, 2021 Category: Hematology Tags: SHORT COMMUNICATIONS Source Type: research
