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The fibrinogen FGG p.Gly242Glu: a rare mutation associated with hypofibrinogenemia
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Letter to the Editor Source Type: research

Activated partial thromboplastin time derivative curves: helpful diagnostic tool in mixing test interpretation
The mixing test is used to evaluate whether prolonged activated partial thromboplastin time (APTT) is due to an inhibitor or a factor deficiency. The coagulation reaction is demonstrated with APTT derivative curves on the ACL TOP series. We aimed to determine the utility of APTT derivative curves in the mixing test process. The plasma of a patient was mixed with normal plasma in a 1 : 1 ratio and APTT assay was performed with SynthASil reagent. We observed roughness, biphasic and shoulder patterns in derivative curves during the mixing test. An extended laboratory investigation revealed a positive lupus anticoagulant, ...
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Short Communication Source Type: research

Clinical and molecular characterization of nine Chinese patients affected by hypofibrinogenemia or dysfibrinogenemia
This study was to elucidate the molecular defects in nine unrelated Chinese patients with hypofibrinogenemia or dysfibrinogenemia. Three fibrinogen genes were amplified by PCR and screened for variants. The identified variants were analyzed by bioinformatics prediction and molecular modeling analysis. Genetic screening disclosed seven different missense mutations, four of which were novel. All of the mutations were expected to impair the protein function/structure as assessed by bioinformatics prediction. This study has increased our knowledge of the mutational spectrum underlying fibrinogen deficiency. (Source: Blood Coag...
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Mutation Report Source Type: research

Conservative management of neonatal cerebral sinovenous thrombosis with coexisting thrombophilia
Neonatal cerebral sinovenous thrombosis (CSVT) comprises approximately 50% of all pediatric-related CSVT. Although guidelines support anticoagulation in pediatric CSVT, the role of anticoagulation in neonatal CSVT remains controversial. This case report details the course of a neonate diagnosed with extensive CSVT and concurrent bilateral thalamic and intraventricular hemorrhage. Due to existing hemorrhage at the time of diagnosis, anticoagulation therapy was not administered. Despite coexisting protein C and antithrombin deficiency, CSVT resolved spontaneously, and neurodevelopmental follow-up after 13 years suggests a go...
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Case Reports Source Type: research

Brachial embolism from mechanical mitral valve thrombosis: multimodality imaging-guided successful thrombolysis
We report the administration of low-dose (25 mg) prolonged infusion (25 h) of tissue-typed plasminogen activator (t-PA) in the treatment of brachial arterial thromboembolism (BAT) secondary to prosthetic valve thrombosis (PVT), under the guidance of multimodality imaging. BAT is a rare disorder with serious consequences. The case of BAT secondary to mitral PVT was successfully treated with low-dose prolonged infusion of t-PA under the guidance of multimodality imaging with upper extremity duplex Doppler ultrasonography, multislice computerized tomographic angiography, coventional angiography and advanced transesophagea...
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Case Reports Source Type: research

Management of heavy menstrual bleeding during direct oral anticoagulant therapy for recurrent venous thromboembolism: a case report
In conclusion, the use of hormonal therapy in VTE women requiring long-term anticoagulation may be an option to control HMB, without further increasing the risk of VTE recurrence. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Case Reports Source Type: research

The effect of obesity on the rate of heparin-induced thrombocytopenia
The objective of this study was to evaluate the association of HIT with obesity in ICU and general medicine patients. We performed a chart review of adult patients within the Methodist Healthcare System, and included patients who had an ELISA and serotonin release assay laboratory tests reported within same hospital admission in which they also had documented receipt of heparin. Obese patients were compared with nonobese patients (BMI  (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Original Articles Source Type: research

An intronic mutation c.6430-3C>G in the F8 gene causes splicing efficiency and premature termination in hemophilia A
This study aimed to investigate the effect of an intronic mutation, c.6430-3C>G (IVS22-3C>G), on pre-mRNA splicing of the F8 gene. We applied DNA and cDNA sequencing in a Chinese boy with hemophilia A to search if any pathogenic mutation in the F8 gene. Functional analysis was performed to investigate the effect of an intronic mutation at the transcriptional level. Human Splicing Finder and PyMol were also used to predict its effect. We found the mutation c.6430-3C>G (IVS22-3C>G) in the F8 gene in the affected boy, with his mother being a carrier. cDNA from the mother and pSPL3 splicing assay showed that the mu...
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Original Articles Source Type: research

Treatment characteristics, efficacy and safety of thrombopoietin analogues in routine management of primary immune thrombocytopenia
We described our experience with TPO-RAs in ITP (chronic, persistent and newly diagnosed ITP) in routine clinical practice. Retrospective series of 100 adult ITP patients was analysed; 41 treated with eltrombopag, 37 with romiplostim and 22 with both. Response-related and safety variables were evaluated. With a median follow-up of 86.5 weeks (interquartile range, 34.3–128 weeks), no differences were found in response rate, time to response, stability of response or response duration based on the type of TPO-RA used. Of all, 25% of patients with newly diagnosed or persistent ITP and 7.2% with chronic responded and mai...
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Original Articles Source Type: research

Anti-Xa activity in oral factor Xa inhibitor-treated patients with atrial fibrillation and a higher risk of bleeding: a pilot study
The number of patients with nonvalvular atrial fibrillation (NV-AF) who require long-term anticoagulation and also have a higher risk of bleeding is increasing. Recently, there is no information regarding real on-treatment anti-Xa activity in patients with NV-AF and a higher risk of bleeding who receive oral factor Xa inhibitors. The aim of this study was to determine trough and peak anti-Xa activity in these patients. This single-centre pilot study enrolled 41 patients with NV-AF and a higher risk of bleeding defined as Hypertension, Abnormal Renal/Liver Function, Stroke, Bleeding History or Predisposition, Labile INR, El...
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Original Articles Source Type: research

Low shear stress upregulates the expression of fractalkine through the activation of mitogen-activated protein kinases in endothelial cells
In this study, EA.hy926 cells were subjected to varying intensity of fluid shear stress for different time durations. Further, mRNA and protein expressions of FKN were assessed by quantitative real-time PCR and Western blotting, respectively. Upregulation of FKN expression, which was induced via activation of mitogen-activated protein kinases signaling pathway under conditions of low shear stress, was studied both in the presence and absence of inhibitors. Low shear stress (∼4.58 dyne/cm2) for more than 1 h promoted FKN expression and activated the extracellular signal-regulated kinase (ERK)1/2, p38, and Jun N-term...
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Original Articles Source Type: research

Increased resistance to tissue plasminogen activator-induced fibrinolysis in healthy subjects from Thailand
There is significant variability in blood coagulation among world populations. In particular, there may exist important differences in regulation of the fibrinolytic system in Asian populations that contribute to diseases of thrombosis and hemostasis. To investigate this issue, we compared fibrinogen concentration, plasma clot formation, and fibrinolytic resistance of healthy Asian subjects from Hat Yai, Songkhla, Thailand (Thai) vs. healthy North American subjects from Seattle, Washington, USA (SEA). Citrated plasma samples were obtained from healthy adult volunteers. Fibrinogen concentration was measured in plasma by the...
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Original Articles Source Type: research

Synergistic inhibitory effect of capsaicin and dihydrocapsaicin on in-vitro platelet aggregation and thromboxane formation
Capsaicinoids, including capsaicin (CAP) and dihydrocapsaicin (DHC), the pungent principles of pepper fruits, individually inhibit in-vitro platelet aggregation. However, their effects, when present together, are not known. The aims of this study were to compare the effects of CAP and DHC alone, and in combination in the ratio that they are found in chilies (∼60% CAP : 40% DHC), on in-vitro platelet aggregation, platelet count and thromboxane B2 (TXB2) formation. The effects of 12.5 and 6.25 μmol/l CAP and DHC individually, and in combination (CAP : DHC, 60 : 40) on arachidonic acid-induced, ADP-induce...
Source: Blood Coagulation and Fibrinolysis - May 11, 2018 Category: Hematology Tags: Original Articles Source Type: research

A retrospective evaluation of the age-adjusted D-dimer versus the conventional D-dimer for pulmonary embolism
The conventional D-dimer cut-off value of at least 500 μg FEU/l has good sensitivity but poor specificity for identifying pulmonary embolism. An elevated age-adjusted D-dimer value (age in years × 10 μg FEU/l) for patients at least 50 years old has been recommended as a better cut-off with adequate sensitivity and improved specificity for identifying pulmonary embolism compared with the conventional value. We retrospectively reviewed 3117 patient encounters in which a D-dimer was ordered. The D-dimer value, age of the patient, and the computed tomography radiology report was evaluated. The sensitivi...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Short Communication Source Type: research

A novel mutation (Tyr503Cys) in a severe factor XI deficiency
The objective is to study a gene mutation (Tyr503Cys) found in a Chinese consanguineous marriage family with inherited factor XI (FXI) deficiency (cross-reacting material positive, type II). The FXI activity and FXI antigen were tested with clotting assay and ELISA, respectively. The FXI gene was amplified by PCR with direct sequencing. ClustalX-2.1-win and three online bioinformatics softwares were used to study the conservatism and harm of the mutation. The proband had reduced FXI: activity at 13%; three members had decreased to about 35%, all of whom had nomal FXI: antigen. DNA sequencing analysis showed the proband car...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Mutation Report Source Type: research

Disseminated intravascular coagulation: is it fact or fancy?
‘Disseminated intravascular coagulation (DIC)’ occurs commonly in critical illnesses such as sepsis, trauma, cancer, and complications of surgery and pregnancy. Mortality is very high. The pathogenesis has been ascribed to tissue factor-initiated coagulation disorder, resulting in disseminated microblood clots that are made of platelets, plasma factors, fibrins, and blood cells. True DIC depletes coagulation factors and consumes platelets, and activates fibrinolysis. ‘DIC’ is assumed to orchestrate thrombocytopenia, microangiopathic hemolytic anemia and hypoxic multiorgan dysfunction syndrome, and c...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Technical Report Source Type: research

Perioperative management of a patient with Glanzmann thrombasthenia undergoing a coronary artery bypass graft surgery: a case report
We report herein the successful perioperative management of a 57-year-old man with a type I Glanzmann thrombasthenia undergoing coronary artery bypass graft surgery and right carotid endarterectomy. The patient suffered from several lesions in the three major coronary arteries and in the right carotid necessitating surgery. Prophylactic human leukocyte antigen (HLA)-matched platelets transfusions were continuous administrated before, and through the immediate perioperative period. Posttransfusion platelet recovery was monitored using flow cytometry to determine the percentage of circulating platelet expressing CD61 (β...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Case Report Source Type: research

Incidence and clinical significance of hyperfibrinolysis during living donor liver transplantation
We evaluated the incidence and clinical significance of hyperfibrinolysis during living donor liver transplantation (LDLT) using viscoelastic coagulation tests. We retrospectively reviewed adult LDLT recipients from February 2010 to February 2015. Hyperfibrinolysis was defined when clot lysis index [LY60 = (MA − A60)/MA × 100, %] was less than 85, where A60 is the clot amplitude at 60 min after maximum amplitude (MA) occurred. Viscoelastic coagulation tests were performed six times (T1: immediately after anesthetic induction, T2: end of preanhepatic phase, T3: 1 h after anhepatic phase, T4: 5 ...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Tissue-type plasminogen activator transgenic rats for evaluating inhibitors of the activated form of thrombin-activatable fibrinolysis inhibitor
No rodent models are currently available for evaluating inhibitors of the activated form of thrombin-activatable fibrinolysis inhibitor (TAFIa) without exogenous supplementation of tissue-type plasminogen activator (tPA). Characterization of tPA transgenic rats as a tool for the nonclinical evaluation of TAFIa inhibitors is the objective of the current study. tPA transgenic rats were subjected to rat models of tissue-factor-induced thromboembolism, FeCl3-induced deep vein thrombosis (DVT) and arterial thrombosis, and tail bleeding. Potato tuber carboxypeptidase inhibitor (PCI), a selective TAFIa inhibitor, was used as an e...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

The primary haemostasis is more preserved in thrombocytopenic patients with liver cirrhosis than cancer
The objective was to compare the haemostatic capacity in different thrombocytopenic patient populations. We evaluated platelet aggregation using impedance aggregometry (Multiplate Analyzer), von Willebrand factor antigen (VWF:Ag), VWF:ristocetin-cofactor activity (VWF:RCo), activated partial thromboplastin time (aPTT), coagulation factor VIII, fibrinogen, and thrombin generation in adult hospitalized patients with platelet count less than 80 × 109/l. Patients either had liver cirrhosis (n = 28), or cancer (n = 169; n = 49 had haematological cancer) with no difference among patients with liver cirrhosi...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

An evaluation of global coagulation assays in myeloproliferative neoplasm
Myeloproliferative neoplasms (MPN) are independent risks for thrombotic events. Routine laboratory tests are inadequate to evaluate the underlying procoagulant state. Global coagulation assays such as thromboelastography, thrombin and fibrin generation may provide better assessment of coagulation activation and thereby of thrombosis risk. Participants with MPN were recruited. Thromboelastography was performed on citrated whole blood while thrombin generation using calibrated automated thrombogram, fibrin generation using overall haemostatic potential assays and P-selectin were quantified on platelet-poor plasma. Thirty-eig...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Evaluation of D-dimer levels in patients with prosthetic valve thrombosis: relationship with thrombus burden and cerebrovascular events
Plasma D-dimer level is an indicator of thrombosis and endogenous fibrinolytic activity. We investigated the association between the D-dimer levels and thrombus burden and cerebrovascular events in patients with obstructive prosthetic valve thrombosis (PVT). This retrospective study included 47 patients with obstructive left-sided PVT and 32 controls in whom PVT was excluded with comprehensive transthoracic and transesophageal echocardiography (TEE). The patient group included 11 aortic, 27 mitral, and 9 aortic and mitral valve PVT patients and the control group included 2 aortic, 25 mitral, and 5 aortic and mitral valve p...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Mean platelet volume and risk of thrombotic and bleeding complications in patients with Philadelphia chromosome negative myeloproliferative neoplasms
This study evaluates the association between MPV and thrombosis and bleeding in MPN. Patients with MPN without prior thrombosis, nonhematologic malignancy or anticoagulant use were included in this retrospective analysis. The primary endpoint was arterial or venous thrombosis. The secondary endpoints were any bleeding and major bleeding. MPV was measured at diagnosis and during the index episode. A total of 135 MPN patients met the inclusion criteria. Over a median follow-up of 6.6 years, 23 patients (15.6%) experienced thrombosis. There was no difference in MPV at diagnosis (8.47 vs. 8.73 fl, P = 0.4) or during the ...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

A decade-long clinical experience on the prophylactic use of activated prothrombin complex concentrate in acquired haemophilia A: a case series from a tertiary care centre
In acquired haemophilia A (AHA), risk for recurrent bleeding exists until the inhibitor is detectable. Thus, patients with persisting inhibitor may benefit from prophylaxis with activated prothrombin complex concentrate (aPCC). Potential thromboembolic complications and cost are also factors to consider. Today, no high level evidence or clear recommendations are available on aPCC prophylaxis in AHA. Recently, a small prospective study demonstrated a favourable outcome with short-term, daily administered aPCC infusion. Here we report a retrospective case series of 19 patients with AHA to demonstrate our practice on aPCC pro...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Identification of haptoglobin as a potential diagnostic biomarker of acute pulmonary embolism
Acute pulmonary embolism is a common disease, which is associated with high mortality and morbidity. There is significant relationship between haptoglobin and pulmonary embolism, however, the usefulness of haptoglobin as a biomarker for the diagnosis of pulmonary embolism remains poorly defined. The aim of the present study was to investigate the change and clinical diagnostic value of haptoglobin in pulmonary embolism. A comparative proteomic analysis was used for clinical screening of serum proteins in 18 patients (9 patients with pulmonary embolism and 9 controls). ELISA was used to validate the dysregulated proteins in...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Association of renin–angiotensin system genes polymorphisms and risk of premature ST elevation myocardial infarction in young Mexican population
The renin–angiotensin system plays an important role in the regulation of blood pressure and the development of coronary artery disease. The aim was to examine the association of the insertion deletion in the angiotensin-converting enzyme gene, M235T and T174M polymorphisms in the angiotensinogen gene with ST elevation acute myocardial infarction (STEAMI) in young Mexican population. We analyzed 242 unrelated patients with STEAMI 45 or less years of age, admitted to a cardiovascular intense care unit, and 242 individuals without STEAMI matched by age and sex, recruited from January 2006 and June 2013. The polymorphis...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Mice with diet-induced obesity demonstrate a relative prothrombotic factor profile and a thicker aorta with reduced ex-vivo function
Classical risk factors such as cholesterol and lipoproteins are currently not sufficient to explain all physiopathological processes of obesity-related vascular dysfunction as well as atherosclerosis and arteriosclerosis. Therefore, the discovery of potential markers involved in vascular dysfunction in the obese state is still needed. Disturbances in hemostatic factors may be involved in the developmental processes associated with obesity-related cardiovascular disorders. We hypothesized that alterations of several hemostatic factors in the obese state could correlate with the function and morphology of the aorta and it co...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Compliance with the 2009 Royal College of Obstetricians and Gynaecologists guidelines for venous thromboembolic disease prophylaxis in pregnancy and postpartum period in Uruguay
This study was conducted at University-Hospital in Uruguay from January 2014 to December 2016. A total of 1035 women were enrolled and stratified in high, intermediate or low risk based on Royal College of Obstetricians and Gynaecologists guidelines. Thromboprophylaxis was recommended for women at intermediate and high risk. Women were followed up to assess symptomatic thromboembolism or haemorrhagic complications. A total of 309 were pregnant and 731 puerperal. Median age was 24 (19–29) years old. Of them, 3.0% (n = 31) were at high risk and 35.4% (n = 366) at intermediate risk. All high-risk women received ...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Complement-coagulation connections
Complement and coagulation are evolutionarily related proteolytic cascades in the blood that are critical for effecting an appropriate innate response to injury that limits bleeding and infection, while promoting healing. Although often viewed as distinct, it has long been recognized that cross-talk likely exists between these pathways. Only recently have molecular links been established. These are providing insights that are revealing opportunities for the development of novel therapeutic strategies to better treat a wide range of thrombotic, inflammatory, immune, infectious, and malignant diseases. In this brief review, ...
Source: Blood Coagulation and Fibrinolysis - April 1, 2018 Category: Hematology Tags: Review Article Source Type: research

Assessing the quality of care for haemophilia at the Yaounde’ reference treatment Centre of Cameroon: Erratum
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Erratum Source Type: research

In silico thrombin and factor Xa generation profiles in adult patients after Fontan operation
Single-ventricle defects are associated with increased risk of thromboembolic events. To analyze the prothrombotic potential in a long-term follow-up on Fontan patients via plasma contribution to thrombin and factor (F)Xa generation profiles. Thrombin and FXa generation was simulated from plasma concentrations of FII, FV, FVII, FVIII, FIX, FX, antithrombin and tissue factor (TF) pathway inhibitor from Fontan patients (n = 48) and healthy controls (n = 34). TF and thrombin–antithrombin complex (TAT) were measured by ELISA. Fontan patients had significantly reduced procoagulant protein concentrations and increa...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Short Communication Source Type: research

Coagulation assays in a case of apixaban overdose
Intentional overdose of apixaban is rare and minimal data exist regarding the usefulness of routinely available laboratory tests to predict drug levels. A 50-year-old man was admitted after ingestion of 200-mg apixaban. Serial blood samples were obtained over a 54-h period for assessment of the fall-off in drug levels using the prothrombin time/international normalized ratio and anti-Xa assays and compared with an apixaban-specific chromogenic assay. The prothrombin time/international normalized ratio and anti-Xa assays correlated with the apixaban level when the drug was in the supratherapeutic range (>130 ng/ml) but...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Case Reports Source Type: research

Low molecular weight heparin versus rivaroxaban in the treatment of venous thromboembolism in gastrointestinal malignancies
We present three patients with high-risk gastrointestinal malignancies complicated by cancer-associated VTE with progression of thrombosis while treated with the oral direct Xa inhibitor rivaroxaban. Upon switching therapy to low molecular weight heparin, we found that these patients had clinical and radiologic improvement of VTE. More studies are needed to evaluate the efficacy of rivaroxaban in high-risk gastrointestinal-VTE. We suggest that in some patients, DOACs may not be sufficient for the treatment of VTEs related to high-risk gastrointestinal malignancies. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Case Reports Source Type: research

Inherited factor II deficiency with paradoxical hypercoagulability: a case report
Congenital deficiency of factor II is a very rare autosomal recessive disorder that can result in a bleeding diathesis. Genotypically, individuals are either homozygous for a defective prothrombin gene or a compound heterozygote with different mutated prothrombin genes inherited from each parent. Phenotypically, it is characterized by either a low production of normal prothrombin or a near-normal production of dysfunctional prothrombin. Treatment is aimed at restoring normally functioning factor II circulating levels to sufficient concentration for hemostasis. Paradoxical thrombosis in patients born from a nonconsanguineou...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Case Reports Source Type: research

Prenatal thrombosis of renal veins and the inferior vena cava in a newborn with double heterozygosity for the factor V Leiden and prothrombin gene G20210A mutations: a case report
We report a case of prenatal thrombosis of the renal veins and the inferior vena cava in a newborn with double heterozygosity for factor V Leiden and prothrombin gene mutations who had persistently impaired renal function requiring chronic peritoneal dialysis. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Case Reports Source Type: research

A novel compound heterozygous mutations in protein C gene causing neonatal purpura fulminans
In this study, we identified a genetic defect in the PROC gene leading to neonatal purpura fulminans. The propositus had very low PC activity (4%) and PC antigen activity (5%). DNA screening of the whole PROC gene revealed two compound heterozygous mutations in exon8 (c.795_796insA) and exon9 (c.1206_1207insG). These two variations led to the compound heterozygous mutations of Gly266Argfs*4 and Pro405Alafs*20, which were inherited from the patient's father and mother, respectively. His older sister is heterozygous for the Gly266Argfs*4 mutation. The inserted nucleotides alter the protein by introducing a stop codon at the ...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Case Reports Source Type: research

Influence of ABO blood group on von Willebrand factor tests in healthy Saudi blood donors
Von Willebrand disease is a common bleeding disorder. The wide variation in von Willebrand factor (VWF) levels between and within normal individuals highlights the clinical challenge of defining its cutoff value. Although studies on the influence of ethnicity on ABO phenotypes and the levels of VWF have been carried out on different ethnicities, there is a lack of such data among Arab population. We aimed to evaluate the correlation of ABO phenotypes with all the parameters of the minimal test panel of VWF including VWF antigen, VWF activity using the ristocetin cofactor and the collagen binding activity assays, and factor...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

Safety and effectiveness of high-dose, weight-based factor VIII inhibitor bypassing activity for warfarin-induced life-threatening bleeding
The objective of this study was to evaluate effectiveness and safety of high-dose, weight-based FEIBA (50 U/kg) vs. frozen plasma alone in this population. This was a matched case–control, multicenter retrospective study including patients who received high-dose, weight-based FEIBA or frozen plasma alone for warfarin-induced life-threatening bleeds matched (1 : 1) based on age and bleed location. Forty-eight patients were included in the analysis (24 FEIBA, 24 frozen plasma). The primary endpoint was time to INR less than 1.5 after administration of FEIBA or frozen plasma. Secondary endpoints include rates of t...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

Thromboelastographic study of the snakebite-related coagulopathy in Djibouti
Hemostasis disorders are one of the major clinical conditions of snakebites and are because of mechanisms which may disrupt vessels, platelets, clotting factors and fibrinolysis. Thromboelastography (TEG) could help to understand these effects in the clinical practice. A retrospective study reports a series of patients presenting a snakebite-related coagulopathy, treated with antivenom and monitored with conventional tests and TEG in a French military treatment facility (Republic of Djibouti, East Africa) between August 2011 and September 2013. Conventional coagulation assays (platelets, prothrombin time, activated partial...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

Systemic lupus erythematosus: disease activity may influence the release of endothelial microparticles?
To evaluate blood-borne endothelial microparticles (EMPs) in women with SLE and correlated these to disease activity as defined by the SLEDAI-2K score. The study takes cross-sectional design. A total of 90 age-matched women were recruited including: G1 (healthy volunteers, n = 30), G2 (women with SLE and low disease activity (SLEDAI-2K score ≤4; n = 30) and G3 (women with SLE and moderate/high disease activity (SLEDAI-2K score>4; n = 30). Blood was collected in 3.2% sodium citrate. Subsequently, the microparticles were purified by ultracentrifugation and labeled with anti-CD51/61 and anti-Annexin-V antibo...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

The effects of transport temperature and time on routine and specialized coagulation assays
Coagulation laboratories have largely stopped transporting whole blood specimens on ice, due to adverse effects on factor VIII, von Willebrand factor, and the prothrombin time. However, it is unknown whether ice should be required or avoided for other coagulation assays. Furthermore, the amount of time that specimens remain stable during transportation at room temperature (RT) is also largely unknown for many coagulation tests. Therefore, this study investigated specimen stability on ice and RT for a comprehensive panel of coagulation tests. One tube of whole blood from each volunteer (n = 22) was centrifuged immediate...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

Prognosis comparisons in acute ischemic stroke patients with thrombolysis and nonthrombolysis therapy: a retrospective study with larger sample size
We compared the short-term, middle-term and long-term primary outcomes between thrombolysis and nonthrombolysis therapy in acute ischemic stroke (AIS) patients. Medical records were collected from patients suffered from AIS between 2010 and 2013 and allocated into either the intravenous recombinant tissue plasminogen activator (as rt-PA group) treatment, or non-rt-PA treatment group (as control group). The primary outcomes included a proportion of patients with favorable outcome [as defined with a modified Rankin Scale (mRS) of 0–1], functional independence (mRS of 0–2) or with bad outcome (mRS of 5–6) at...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

An ex-vivo model of shear-rate-based activation of blood coagulation
The study presents a model of shear-stress-based platelet activation. Twenty-eight patients (22 free from anticoagulants and major antiplatelet agents, and six under the effects of P2Y12 platelet inhibitors) participated. The main purpose was to verify the hypothesis that a model of shear-dependent blood activation does not require artificial activators to trigger clot formation. Whole blood collected from the patients received platelet function tests [ADPtest and thrombin receptor-activating peptide (TRAP)test] and was tested with a cone-on-plate viscosimeter at a shear rate of 100 s−1. Changes in blood viscosity ...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

Association of hyperglycemia, systolic and diastolic hypertension, and hyperthermia relative to baseline in the acute phase of stroke with poor outcome after intravenous thrombolysis
In the acute phase of ischemic stroke, the trend of some physiological variables, such as blood glucose (BG), blood pressure, and body temperature (BT), might influence outcome. We aimed to assess the association of hyperglycemia, systolic and diastolic hypertension, and hyperthermia relative to baseline BG, systolic blood pressure, diastolic blood pressure and BT, respectively, in the first 12 h with poor outcome after intravenous thrombolysis (IVT). We conducted a retrospective analysis of data prospectively collected from 200 consecutive anterior ischemic stroke patients treated with IVT. Outcome measures were no neur...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

Thrombin potential and traditional coagulation assay: are they useful in exploring recurrent pregnancy loss risk?
In this study, we used the global coagulation assay, calibrated automated thrombography and traditional coagulation assay to search for a possible underlying hypercoagulable status in women with history of RPL compared with uneventful pregnancy women. Thrombin generation, Fibrinogen, factor VIII (FVIII), Plasminogen Activator Inhibitor-1 (PAI-1) and von Willebrand factor levels were analyzed in 92 not pregnant unexplained RPL and 64 uneventful pregnancy women. In RPL women, significantly higher fibrinogen, FVIII and PAI-1 levels, and thrombin generation with respect to those observed in uneventful pregnancy women were foun...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

Congenital hypofibrinogenemia in pregnancy: a report of 11 cases
To investigate the obstetrical outcomes and plasma concentrations of fibrinogen (FIB) in patients with congenital hypofibrinogenemia in pregnancy, 11 cases with hypofibrinogenemia in pregnancy were analyzed retrospectively. The demographic database, bleeding tendency, plasma levels of FIB throughout pregnancy, peripartum management, as well as, maternal and neonatal outcomes were assessed. FIB levels in our patients remained relatively stable throughout the pregnancy. The mean FIB levels during the three trimesters of pregnancy were 75.7 ± 43.9 (25–148), 67.6 ± 33.7 (22–146), and 77.9 &p...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

Thrombin generation estimates the anticoagulation effect of direct oral anticoagulants with significant interindividual variability observed
Direct oral anticoagulants (DOACs) are increasingly being used, primarily due to their drug stability and patient convenience. Although these drugs have been evaluated to be well tolerated in numerous clinical trials, their impact on in-vivo anticoagulation effect, variability and therapeutic drug level remains unknown. Hence, we aim to study the effect and variability of DOACs on thrombin generation via the calibrated automated thrombogram. Anonymized coagulation specimens from outpatients on warfarin were collected. Pooled normal plasma samples were spiked with increasing concentrations of dabigatran, rivaroxaban and api...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

Efficacy of treatment immune thrombocytopenic purpura in pregnancy with corticosteroids and intravenous immunoglobulin: a prospective follow-up of suggested practice
The current study is performed to assess a routine for treatment of immune thrombocytopenic purpura in pregnancy. A prospective programme for monitoring and treatment with intravenous immunoglobulin or cortisone in pregnancies with immune thrombocytopenic purpura was suggested to all delivery units in Sweden. Treatment should be avoided if platelet counts were more than 20 × 109/l during pregnancy with no bleeding complications and with a target of 100 × 109/l at delivery. Descriptive statistics and logistic regression analysis were used. Seventy-five pregnancies were followed; treatment was given in 29...
Source: Blood Coagulation and Fibrinolysis - February 9, 2018 Category: Hematology Tags: Original Articles Source Type: research

Comparison of human coagulation factor VIII expression directed by cytomegalovirus and mammary gland-specific promoters in HC11 cells and transgenic mice: Retraction
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - January 1, 2018 Category: Hematology Tags: Retraction Source Type: research

Oral anticoagulation cost in primary antiphospholipid syndrome: comparison between warfarin and hypothetical rivaroxaban
The objective of the study is to perform a cost comparison of 1 year of oral anticoagulation management with warfarin vs. hypothetical rivaroxaban (RRX) in thrombotic primary antiphospholipid syndrome. Longitudinal study on 20 primary antiphospholipid syndrome patients followed-up for 1 year after anticoagulation stabilisation with warfarin: a dedicated software calculated number of clinic visits, time in therapeutic range and warfarin consumption for each patient; for RRX, we considered a visit every 3 months (baseline done in hospital before attending anticoagulant clinic); knowing the cost of both anticoagulants, the la...
Source: Blood Coagulation and Fibrinolysis - January 1, 2018 Category: Hematology Tags: Short Communication Source Type: research