Impact of obesity on heparin-induced thrombocytopenia in cardiac surgery patients
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: LETTER TO THE EDITOR Source Type: research

Thromboelastography in the management of snakebite-induced coagulopathy: a case series and literature review
We report on three cases of North American crotaline envenomation with subsequent TEG analysis. Our results show that TEG analysis does relate the same information as traditional monitoring parameters. Although only in an isolated case, the TEG analysis potentially revealed a fibrinolytic process prior to the same process being apparent with traditional monitoring. Future research is necessary to identify the role of TEG in crotaline snakebite management. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: CASE REPORTS Source Type: research

Management of dabigatran after overdosage: two case reports and suggestions for monitoring
We describe two different cases with very high overdosage in which PCC or idarucizumab was used to reverse the effect of dabigatran etexilate. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: CASE REPORTS Source Type: research

A novel type 2N VWF gene mutation: a case report
We present a case that highlights the difficulties of recognizing this diagnosis, the role of genetic testing, and the identification of a 2N variant that has not been previously described. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: CASE REPORTS Source Type: research

Reliability of the portable coagulometer qLabs to accurately measure the activated thromboplastin time and international normalized ratio: a prospective study in critically ill patients
The current prospective study was aimed at investigating whether a portable coagulometer (qLabs) can be used to reliably monitor activated thromboplastin time (aPTT) and international normalized ratio (INR) in critically ill patients, as compared with standard central laboratory measurement. Both precision and accuracy of INR and aPTT measured by qLabs were assessed in this observational study by finger prick group (N = 30 patients) and blood droplet group from central venous catheter drawn (N = 60). For accuracy, clinical agreement percentage was ±0.3 for INR and ±10 s for aPTT. Precision of INR me...
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Optimizing outcome measurement with murine ferric chloride-induced thrombosis
The murine FeCl3 model is a widely used model for studying arterial thrombosis, yet provides limited information from each mouse, often only a single time point for the onset of occlusion (defined as the time to occlusion; TTO). To optimize data from the murine ferric chloride model of thrombosis. FeCl3 injury was induced in the carotid arteries of wild-type and Factor IX (FIX) knockout mice, with infusion of recombinant FIX (rFIX) to normalize FIX deficiency at various times around FeCl3 injury. The TTO was recorded as a percentage of baseline flow as occlusion continued to zero flow, with identification of reflow events....
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Effect of ABO blood group on haemostatic parameters in severe haemophilia A patients performing acute moderate-intensity exercise
The primary objective was to assess the effect of ABO blood group on von Willebrand factor (VWF) rise induced by four bouts of moderate-intensity physical activity, on pharmacokinetics of a B-domain-deleted recombinant FVIII (BDD-rFVIII), and haemostatic parameters in severe haemophilia A patients with a null mutation. The secondary objective was to compare the response to exercise according to infused product type in a subgroup of patients who previously participated to the same exercise protocol, while treated with a full length recombinant FVIII (FL-rFVIII). Twenty patients had two visits (rest and exercise). Blood samp...
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Genotyping of five Pakistani patients with severe inherited factor X deficiency: identification of two novel mutations
Congenital factor X deficiency is a rare coagulation defect characterized by variable bleeding tendency. The aim of the study was to give a first insight of F10 gene mutations in Pakistani probands. Direct sequencing and/or next-generation sequencing was performed on the coding regions, boundaries and 5’ and 3’ untranslated regions of the F10 gene in five severe factor X-deficient patients from Pakistan. All patients were born from consanguineous marriages and displayed FX:C levels below 2%. Sequencing revealed five different substitutions, including three previously reported p.Ala15Asp, p.Gly406Ser, and p.Gly4...
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

L-Carnitine as an additive in Tyrode's buffer during platelet storage
This study contributes towards the development of better storage solutions for platelets. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Novel isomeric metabolite profiles correlate with warfarin metabolism phenotype during maintenance dosing in a pilot study of 29 patients
For this pilot study, we leveraged metabolite patterns for warfarin patients to more accurately assess clinically relevant differences in drug metabolism. We tested our hypothesis that plasma metabolite levels correlate with the influence of clinical factors on R-warfarin and S-warfarin metabolism (warfarin metabolic phenotype). We recruited 29 patients receiving a maintenance dose and testing within targeted therapeutic range. We determined their CYP2C9 and vitamin K epoxide reductase genotype and profiled 14 isomeric forms of warfarin and its metabolites. We employed three novel types of clearance ratios using analyte le...
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Thrombosis in pediatric malignancy: a review and future perspectives with focus on management
Venous thromboembolism (VTE) result in significant morbidity and mortality in children with cancer. The cause of VTE in children with cancer is multifactorial and includes genetic predisposition (thrombophilia), disease-related factors, and treatment-related factors including use of central venous catheter (CVC), surgery, and chemotherapy. This review aims to examine current knowledge regarding the incidence, risk factors, clinical manifestation, evaluation, prevention, and management of thromboembolic events in children with cancer. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: REVIEW ARTICLES Source Type: research

Thrombogenesis and thrombotic disorders based on ‘two-path unifying theory of hemostasis’: philosophical, physiological, and phenotypical interpretation
Hemostasis, endowed to human to protect lives, is a process of logical blood clotting system to prevent blood loss in vascular injury. However, the notion that deadly thrombosis occurs as a result of normal hemostasis in intravascular injury could encounter with conceptual skepticism because the term ‘thrombosis’ automatically conjures up as serious disease. According to ‘two-path unifying theory’, normal hemostasis is initiated only by vascular injury through activated unusually large von Willebrand factor (ULVWF) path and/or activated tissue factor (TF) path. When these two equally important paths...
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: REVIEW ARTICLES Source Type: research

Hemostasis based on a novel ‘two-path unifying theory’ and classification of hemostatic disorders
Hemostasis is the most important protective mechanism for human survival following harmful vascular damage caused by internal disease or external injury. Physiological mechanism of hemostasis is partially understood. Hemostasis can be initiated by either intravascular injury or external bodily injury involving two different levels of damage [i.e., limited to the endothelium or combined with extravascular tissue (EVT)]. In intravascular injury, traumatic damage limited to local endothelium typically is of no consequence, but disease-induced endothelial damage associated with systemic endothelial injury seen in sepsis and ot...
Source: Blood Coagulation and Fibrinolysis - October 17, 2018 Category: Hematology Tags: REVIEW ARTICLES Source Type: research

Thromboelastographic analysis of haemostasis in preeclamptic and normotensive pregnant women
Pregnancy is a state of heightened coagulation, exacerbated in pathological conditions such as preeclampsia. We evaluated the role of thromboelastography (TEG), compared with standard haemostasis tests, in identifying in haemostatic alterations in normotensive pregnancies and pregnancies complicated with preeclampsia. Standard haemostasis tests and TEG were performed on 28 normotensive women and 31 with preeclampsia at delivery, 6 weeks and 6 months postpartum. Results were compared between patient groups, and at different collection times. Standard haemostasis tests failed to reveal consistent differences in haemostatic f...
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Short Communications Source Type: research

Validation of the STA-Liatest DDi assay for exclusion of proximal deep vein thrombosis according to the latest Clinical and Laboratory Standards Institute/Food and Drug Administration guideline: results of a multicenter management study
Recommended strategy for venous thromboembolism (VTE) diagnosis includes the use of sensitive D-dimer (DDi) assays along with pretest probability (PTP) assessment. The Clinical and Laboratory Standards Institute (CLSI) recently issued a guideline (US FDA endorsed) on DDi in VTE exclusion. Such guideline specifies the ideal D-dimer assay characteristics and target population. Demonstrate STA-LiatestD-Di performance combined with a PTP score for proximal deep vein thrombosis (pDVT) exclusion in a CLSI compliant study. International, multicenter, prospective nonrandomized, noninterventional clinical outcome management study c...
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Short Communications Source Type: research

Successful treatment of massive pulmonary embolism with low-dose tissue plasminogen activator after meniscus surgery
Pulmonary embolism is a life-threatening disease which represents with high mortality and morbidity. Systemic fibrinolysis is the first-line recommended therapy in hemodynamically instable patients with massive pulmonary embolism. Systemic fibrinolytic treatment remains controversial for pulmonary embolism after surgery due to high risk of hemorrhage. Herein, we report a 49-year-old man with massive pulmonary embolism after meniscus surgery, successfully treated with low-dose prolonged infusion of tissue type plasminogen activator. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Case Report Source Type: research

Comparison of a new criteria for sepsis-induced coagulopathy and International Society on Thrombosis and Haemostasis disseminated intravascular coagulation score in critically ill patients with sepsis 3.0: a retrospective study
This study included 252 patients. The International Society on Thrombosis and Haemostasis (ISTH) disseminated intravascular coagulation (DIC), modified ISTH-DIC, and SIC scores were higher among nonsurvivors (P  (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Original Articles Source Type: research

Neonates born to mothers with immune thrombocytopenia: 11 years experience of a single academic center
A major problem associated with immune thrombocytopenic purpura (ITP) in pregnancy is neonatal thrombocytopenia. We analyzed newborns born to mothers with ITP and examined predictive factors for thrombocytopenia. This retrospective study was performed in a single academic center from January 2007 to January 2018. Pregnant women with ITP and their babies are included. All neonates had a complete blood count and cranial ultrasound (USG) performed. Twenty seven neonates of 22 mothers were evaluated. A total of 23 (85%) of neonates were thrombocytopenic ( (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Original Articles Source Type: research

Wdr-1 is essential for F-actin interaction with focal adhesions in platelets
Wdr-1, an actin interacting protein, enhances cofilin's capacity to accelerate depolymerization of F-actin filaments. Wdr-1-deficient mice have impaired hemostasis due to defective inside-out integrin signaling in platelets. Here, we studied the role of Wdr-1 on outside-in signaling necessary for retraction of the clot and platelet spreading. Outside-in signaling was assessed by fibrin clot retraction assay and by adhesion and spreading of unstimulated platelets on fibrinogen substrate. The spatial distribution of actin, cofilin-1 and Wdr-1 were determined by immunofluorescence microscopy. Interaction of F-actin with focal...
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Original Articles Source Type: research

Thromboelastometric prediction of mortality using the kinetics of clot growth in critically ill septic patients
The objective of this study is to evaluate a new ROTEM clot velocity parameter representing clot formation kinetics in septic ICU patients. We hypothesized that this time-based parameter was significantly different in critically ill septic survivors vs. nonsurvivors. This retrospective observational study included severe sepsis and septic shock patients having a minimum of two organ dysfunctions by Sequential Organ Failure Assessment criteria (study group), and a control group of healthy adults without infection . ROTEM with EXTEM activation was performed in all groups. The time from maximal clot formation velocity to zero...
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Original Articles Source Type: research

Venous thromboembolism risk associated with ABO, F11 and FGG loci
The current study aims to evaluate, for the first time in the Portuguese population, the association with venous thromboembolism (VTE) of five well known and replicated VTE-associated single nucleotide polymorphisms (SNPs) in genes ABO, F11 and FGG. A population sample of 96 cases of VTE, without strong or moderate inherited or noninherited predisposing factors, and 148 healthy controls were analyzed for variants in genes ABO (rs2519093; rs8176719), F11 (rs2036914; rs2289252) and FGG (rs2066865). SNPs were genotyped by real-time PCR with TaqMan probes or by PCR–restriction fragment length polymorphism. Logistic regre...
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Original Articles Source Type: research

Rivaroxaban and dabigatran did not affect clotting profiles in plasma reconstituted with varying levels of autologous platelets to the same degree as heparin when evaluated using thromboelastography
Conclusion All anticoagulants tested compromised coagulation at specific platelet concentration thresholds. Rivaroxaban and dabigatran had reduced impact on clot formation at low-platelet concentrations compared with heparinoids, suggesting that the factor-specific inhibitors may be more favorable than traditional heparin-based treatment of thromboembolism in the presence of thrombocytopenia. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Original Articles Source Type: research

Evaluation of early musculoskeletal disease in patients with haemophilia: results from an expert consensus
Early joint damage in patients with haemarthrosis often escapes diagnosis because of insufficient investigation of biomechanical changes. Arthropathy in haemophilia requires complex assessment with several tools. Considering the increased emphasis on an integrated approach to musculoskeletal (MSK) outcomes, re-evaluation of MSK assessment to address individual patient needs is warranted. To advise on the optimal use of current assessment tools and strategies for tailored MSK evaluation in patients with haemophilia. A panel of experts in haemophilic arthropathy evaluated internationally recognized assessment tools through p...
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Original Articles Source Type: research

Simvastatin reverses multiple myeloma serum-induced prothrombotic phenotype in endothelial cells via ERK 1/2 signalling pathway
This study supports the beneficial effects of simvastatin on multiple myeloma serum-induced endothelial haemostatic imbalance, which suggests that simvastatin may serve as a new and appropriate antithrombotic approach for multiple myeloma patients. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Original Articles Source Type: research

Clinical and genetic factors influencing acenocoumarol dosing: a cross-sectional study
Coumadin oral anticoagulants are widely used in multiple clinical scenarios. Their narrow therapeutic range and a dosing strategy based on ‘a posteriori’ algorithms, pose them as an interesting group for prediction modelling research. Extensive literature explaining the association between clinical and genetic variables with the dose of warfarin have been published. Limited information exists regarding these factors and acenocoumarol dosing. The aim of the study is to explain through clinical/genetic variables, the weekly dose of acenocoumarol necessary for achieving stable anticoagulation status. We performed ...
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Original Articles Source Type: research

Leu33Pro (PlA) polymorphism of integrin beta3 modulates platelet Src pY418 and focal adhesion kinase pY397 phosphorylation in response to abnormally high shear stress
Conclusion Taken together, our data indicates an important role of αIIbβ3 and shows that Leu33Pro polymorphism modulates the integrin-mediated Src and FAK signaling in platelets in response to shear stress. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Original Articles Source Type: research

Ibrutinib-related bleeding: pathogenesis, clinical implications and management
Ibrutinib is the first drug of a new family of Bruton's tyrosine kinases (Btk)-inhibiting agents, which have proved to be useful for the treatment of several B-cell lymphoid malignancies. This drug is associated to an increased bleeding risk from initial clinical trials especially in association with warfarin. Although Btk plays an important role in platelet signalling, increased bleeding tendency in patients on ibrutinib is more complex than Btk inhibition alone and is because of several antiplatelet mechanisms, namely inhibition of Btk and Tec kinases, which play a key role in platelet activation downstream of the collag...
Source: Blood Coagulation and Fibrinolysis - August 17, 2018 Category: Hematology Tags: Review Article Source Type: research

Homozygous congenital factor VII deficiency with a novel mutation, associated with severe spontaneous intracranial bleeding in a neonate
Conclusion Early diagnosis and treatment of congenital FVII deficiency can be crucial. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Mutation Report Source Type: research

Anticoagulation in a patient with antiphospholipid syndrome and a mechanical heart valve: a case study
A case report of a patient with antiphospholipid syndrome (APS) with positive lupus anticoagulant and a mechanical mitral valve replacement (MVR) who presented with a thrombosis and difficult-to-control INRs. Current literature on anticoagulation options for patients who present with these conditions as well as alternate laboratory assays are reviewed. APS can present a unique complexity and challenge to appropriate anticoagulation on warfarin. Although there are emerging data regarding the use of novel oral anticoagulants to treat this condition, they do not justify their use in a patient with APS and an MVR because of a ...
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Case Reports Source Type: research

Successful treatment of severe menorrhagia at menarche with recombinant factor VIIa in an adolescent girl with type III von Willebrand's disease
We present an adolescent case with type III vWD who had a menorrhagia at menarche that was refractory to the standard treatment and ultimately was treated with rFVIIa successfully. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Case Reports Source Type: research

Immune tolerance induction rescue with turoctocog-alfa in a poor risk haemophilia A inhibitor young child: the history of a success
We report a successful case of a poor risk (titre of inhibitor at start of ITI > 10 BU/ml, peak titre on ITI > 200 BU/ml,>2 years since the inhibitor diagnosis) haemophilia A child treated with a high-dose regimen (200 UI/kg/day) turoctocog-alfa after a failed first-line ITI with octocog-alfa lasting 29 months. At 22 months of ITI-R, the inhibitor titre was undetectable, the FVIII recovery was 74%, of the expected level and the FVIII half-life more than 7 h. A complete successful ITI-R was then achieved with turoctocog-alfa. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Case Reports Source Type: research

Aberrant phosphorylation of signal transducer and activator of transcription 3 protein of the CD4+ T cells in patients with primary immune thrombocytopenia
Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by decreased platelet count of which dysfunctional cellular immunity in its pathogenesis. Signal transducer and activator of transcription 3 (STAT3) is critical for the differentiation of T cells. The present study was aimed to investigate the STAT3 protein phosphorylation of CD4+ T cells in ITP patients. Fourteen patients of newly diagnosed ITP with complete remission (R group) and other 15 newly diagnosed ITP patients with nonresponse (N group) after corticosteroids therapy were included. Sixteen healthy human volunteers were served as normal c...
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Application of tranexamic acid and diluted epinephrine in primary total hip arthroplasty
Tranexamic acid (TXA) and diluted epinephrine (DEP) has been reported to be an efficient and well tolerated way for reducing blood loss in total hip arthroplasty (THA). This meta-analysis was designed to compare the effectiveness of combination application of TXA with DEP in primary THA. The following electronic databases were searched, including PubMed, EMBASE, Web of Science, the Cochrane Library, China National Knowledge Infrastructure and Google Search Engine, for published studies involving the TXA with DEP in primary THA. All randomized controlled trials (RCTs) were included. Statistical analysis was assessed using R...
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

The effects of Plasma-Lyte 148 solution on blood coagulation: an in-vitro, volunteer study using rotational thromboelastometry
The current study aimed to measure the effects of Plasma-Lyte 148 solution on the blood coagulation profile according to the hemodilution level using rotational thromboelastometry (ROTEM) tests. Venous blood was collected from 12 healthy volunteers and divided into four specimen bottles, which were diluted at different levels with Plasma-Lyte 148 (0, 20, 40, and 60%). Following this, ROTEM tests were performed on the study samples. We found that as the hemodilution level increased, the ROTEM values showed a hypocoagulable pattern. The change rate of the maximum clot firmness (MCF) of INTEM was greater in the 40 (P = 0....
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Association of hereditary antithrombin deficiency with intrauterine growth restriction
Antithrombin is a major suppressor of thrombin, factor Xa and blood coagulation. Inherited antithrombin deficiency is rare and is considered among the common causes of inherited thrombophilia. The relationship between antithrombin and IUGR, is questionable. The aim of this study was to trace the relationship between antithrombin deficiency and the intrauterine weight reduction and neonatal morbidity. The study was conducted on 55 full-term neonates (including 25 baby boys and 30 baby girls), all were admitted to Neonatal Intensive Care Unit in Cairo University Hospitals with IUGR; and another 110 healthy full-term neonates...
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Detection of high-risk thrombophilia with an automated, global test: the Coagulation Inhibitor Potential assay
The diagnosis of thrombophilia is a cost-consuming and time-consuming process, as each defect should be separately investigated. The Coagulation Inhibitor Potential (CIP) assay is a promising new global test, sensitive for most of the hereditary thrombophilias, developed for manual methodology. We adapt the original method to an optical coagulation analyser. By this automation, the test will be easier, faster and more precise, and it also allows carrying out 18 measurements simultaneously. The CIP assay was performed in 126 healthy subjects and 193 patients with different types of hereditary thrombophilia conditions. Detec...
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Genetic causes of resistance to vitamin K antagonists in Polish patients: a novel p.Ile123Met mutation in VKORC1 gene
Mutations in the genes encoding vitamin K epoxide reductase complex subunit 1 (VKORC1) and cytochrome P450 2C9 (CYP2C9) largely contribute to the inter-individual variations in vitamin K antagonists (VKAs) dose requirements. Up to 50% of the dosage variability can be explained by genetic polymorphisms in these genes. We sought to identify the mutations responsible for VKA resistance in a series of Polish patients. Of the 607 patients treated with VKA, 35 (6%) individuals with the VKA resistance defined as a daily dose of acenocoumarol more than 8 mg (n = 15, 43%) or warfarin more than 10 mg (n = 20, 57%) were s...
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Cardiovascular diseases in congenital prekallikrein deficiency: comparison with other chance-associated morbidities
To compare the prevalence of cardiovascular diseases with other chance-associated morbidities in patients with congenital prekallikrein deficiency. Patients with prekallikrein deficiency were gathered from two time unlimited PubMed searches and from personal files. Inclusion criteria were prekallikrein level less than 15% of normal; correction of aPTT on long incubation times; prolonged aPTT corrected by normal plasma or serum; normal prothrombin time and normal FXII and FXI. Acquired forms were excluded. Out of 106 patients, we have found that 45 patients had at least one chance-associated defect or morbidity at the time ...
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

Can the plasmaderived factor VIII still play a role in the treatment of acquired hemophilia A at the time of new drugs?
Bypassing agents are the first-line therapy in the treatment of acquired hemophilia A (AHA), but not the only one. Other options as recombinant porcine factor VIII or plasmaderived concentrates (pdFVIII) are available to clinicians. Aim of this study was to evaluate whether the pdFVIII can still play a role in the treatment of AHA, and which patients could benefit from this therapy. All patients with AHA, presenting severe cardiovascular comorbidities, and treated with pdFVIII with or without von Willebrand factor (vWF), referred to two different hospitals, were initially considered. Eight patients were studied and divided...
Source: Blood Coagulation and Fibrinolysis - July 1, 2018 Category: Hematology Tags: Original Articles Source Type: research

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