Coagulation factor XIII-A and activated FXIII-A decrease in some deep vein thrombosis patients following catheter-directed thrombolysis
The objective of the study was to assess the effect of tissue plasminogen activator administered during catheter-directed thrombolysis (CDT) on coagulation factor XIII (FXIII). Thrombolytic therapy carries significant risks, such as life-threatening bleeds. The mechanisms responsible for major bleeds and intracerebral hemorrhages during thrombolysis are not fully understood. Activated FXIII (FXIII-A*) lies at the intersection of coagulation and fibrinolysis. Using purified proteins and blood collected from nine deep vein thrombosis patients undergoing CDT, the stability of FXIII-A* and FXIII were measured immediately befor...
Source: Blood Coagulation and Fibrinolysis - May 15, 2019 Category: Hematology Tags: SHORT COMMUNICATION Source Type: research

A case-report of successful use of thromboelastography to manage anticoagulation therapy in a patient with concomitant catastrophic antiphospholipid syndrome and heparin-induced thrombocytopenia
Catastrophic antiphospholipid syndrome (CAPS) is a severe but rare form of antiphospholipid syndrome (APS) that results in multiple thrombosis of multiple organs within a week [1]. Similarly, heparin-induced thrombocytopenia (HIT) has been associated with severe and life-threatening thrombosis, both conditions mediated by an autoimmune disorder resulting in a highly thrombotic state [2,3]. Both conditions requiring aggressive therapeutic anticoagulation when coinciding CAPS complicated by HIT presents a therapeutic challenge. Current recommendations advocate for the use of anti-Xa activity monitoring in the setting of APS ...
Source: Blood Coagulation and Fibrinolysis - May 15, 2019 Category: Hematology Tags: CASE REPORTS Source Type: research

Recombinant Von Willebrand factor concentrate in 2A Von Willebrand disease: comparison to plasma-derived Von Willebrand factor concentrate therapy
We describe a patient with Type 2A VWD who had persistent refractory epistaxis despite treatment with PD-VWFC. We describe differences in VWF multimeric composition and Factor VIII (FVIII) levels after plasma-derived and rVWF concentrates. Despite similar VWF levels, VWF multimeric composition after PD-VWFC remained abnormal while it corrected with rVWFC. Post-PD-VWFC, high levels of FVIII were seen, which were not observed after rVWFC. Recombinant VWFC may offer some advantages over PD-VWFC. This finding needs to be confirmed in larger studies. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - May 15, 2019 Category: Hematology Tags: CASE REPORTS Source Type: research

Case report of two siblings with type 2A von Willebrand disease involving a novel mutation within the calcium-binding site of the A2 domain of von Willebrand factor
We report a novel VWF variant, a heterozygous single nucleotide change, c.4493A>G, occurring at the p.Asp1498 residue of the calcium-binding site of the A2 domain in two sisters with type 2A von Willebrand disease. Modest increase in the VWF propeptide/VWF:Ag ratio (2.4 and 2.7) supports increased clearance of VWF. A literature review provided insight into the integral role of p.Asp1498 residue in calcium-binding and its role in the stabilization of other residues including p.Arg1597 and p.Asn1602. Studies done by other groups on engineered mutations involving p.Asp1498 reported increased susceptibility to ADAMTS13 prot...
Source: Blood Coagulation and Fibrinolysis - May 15, 2019 Category: Hematology Tags: CASE REPORTS Source Type: research

Comparison of clot-based and chromogenic assay for the determination of protein c activity
In conclusion, we recommend the chromogenic method as the assay of choice, which is also recommended by the College of American Pathologist Consensus Study over activated partial thromboplastin time-based assay. We have shown here that despite a good correlation between the two techniques, there is a difference as highlighted by the difference plots. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - May 15, 2019 Category: Hematology Tags: TECHNICAL REPORT Source Type: research

Analysis of apoptotic, platelet-derived, endothelial-derived, and tissue factor-positive microparticles of children with acute lymphoblastic leukemia during induction therapy
Conclusion Our study demonstrated that children with ALL have increased levels of apoptotic, platelet-derived, endothelial-derived, and tissue factor-positive microprticles during induction therapy. Further studies are needed in larger groups of patients in order to evaluate the risk of elevated microprticles for development of thromboembolism during induction therapy period in children with ALL. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - May 15, 2019 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Recombinant human prothrombin (MEDI8111) combined with fibrinogen dose-dependently improved survival time and reduced blood loss in a porcine model of dilutional coagulopathy with uncontrolled bleeding
Uncontrolled bleeding due to trauma and coagulopathy is an area with high unmet medical need and high mortality rate. Treatment recommendations focus on transfusion of blood components while optimal therapy to improve coagulation remains to be established. The haemostatic effect of 2, 4 and 8 mg/kg recombinant prothrombin (MEDI8111) co-administered with 100 mg/kg fibrinogen (n = 7–8) was investigated in a porcine model of dilutional coagulopathy with uncontrolled bleeding. Vehicle (n = 11), fibrinogen alone (100  mg/kg , n = 15) were included as controls. Dilutional coagulopathy was induced by rep...
Source: Blood Coagulation and Fibrinolysis - May 15, 2019 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Blockade of T-cell immunoglobulin and mucin domain-containing molecule 3 aggravates T-helper cell 1 polarization in immune thrombocytopenia
An increased T-helper cell (Th) 1/Th2 ratio in the peripheral blood has been proposed to correlate with the disease activity of immune thrombocytopenia (ITP). T-cell immunoglobulin and mucin domain-containing molecule 3 (TIM-3) is a Th1-associated cell surface molecule that regulates Th1 responses and promotes tolerance. Consequently, we aimed to determine whether the regulation of TIM-3 expression is likely to be a promising therapeutic approach for ITP. In the present study, we investigated the immunomodulatory activities of TIM-3 in human peripheral blood mononuclear cell (PBMC) cultures. Levels of interferon-gamma (IFN...
Source: Blood Coagulation and Fibrinolysis - May 15, 2019 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Involvement of antifactor VIII autoantibodies specificity in the outcome of inhibitor eradication therapies in acquired hemophilia a patients
We hypothesized that inhibitor specificity may predict the outcome of antifactor VIII autoantibodies eradication treatment in acquired hemophilia A. Our objective was to analyze the association between factor VIII domains recognized by inhibitors and outcome of the immunosuppressive therapies (ISTs) in a prospective, observational study. 16 patients were recruited. Inhibitor specificities were assessed at diagnosis and throughout the study. Their association with IST outcome was addressed. First-line IST succeeded in 56% of patients. Inhibitors reacted mainly with light chain domains (69%) and/or the A2 domain (44%). 31% i...
Source: Blood Coagulation and Fibrinolysis - April 1, 2019 Category: Hematology Tags: SHORT COMMUNICATION Source Type: research

Compartment syndrome of the forearm with life-threatening bleeding after fasciotomy as the presenting sign of postpartum acquired hemophilia A: a case report
We report a rare case of a compartment syndrome of the forearm in a 30-year-old woman 2.5 months postpartum as the presentation of pregnancy-associated AHA. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - April 1, 2019 Category: Hematology Tags: CASE REPORT Source Type: research

Accelerating recovery from acute hemarthrosis in patients with hemophilia: the role of joint aspiration
This study shows that joint aspiration under hemostatic cover and in strictly aseptic conditions is a well-tolerated technique that makes the recovery of acute hemarthrosis of hemophilic patients faster. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - April 1, 2019 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Thrombocytopenia in well small for gestational age neonates
Thrombocytopenia has been closely associated with small-for-gestational-age neonates (SGA; birthweight less than tenth percentile) admitted to the special care nursery or neonatal ICU. It is unclear if ‘well’ SGA neonates experience the same incidence of thrombocytopenia in the SGA population as compared to sick neonates. We conducted a retrospective cohort study from a health network in Melbourne, Australia, between 2012 and 2015 to identify SGA neonates (≥ 35 weeks’ gestation at birth) that were otherwise well. Neonates with at least one platelet count within 7 days of life were matched to appropriat...
Source: Blood Coagulation and Fibrinolysis - April 1, 2019 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Efficacy of direct oral anticoagulants on left ventricular thrombus
Left ventricular thrombus (LVT) is a commonly seen complication of myocardial infarction and it also can be seen because of hypertrophic cardiomyopathies, nonischemic dilated cardiomyopathies, malignancies, and so on. Guidelines suggest the use of warfarin for left ventricular thrombi, but recent case reports show that direct oral anticoagulants (DOACs) are beginning to be used for this complication. DOACs are strong alternatives for warfarin because of their efficacy and safety even though there is no randomized controlled trial that proves the effect of DOACs against LVT. In this article, we gather the case reports of DO...
Source: Blood Coagulation and Fibrinolysis - April 1, 2019 Category: Hematology Tags: REVIEW ARTICLES Source Type: research

Rivaroxaban and the EINSTEIN clinical trial programme
Rivaroxaban, a direct oral anticoagulant, is widely used for the treatment of venous thromboembolism (VTE) in adult patients. The approval of rivaroxaban for the treatment of deep vein thrombosis and pulmonary embolism and the extended secondary prevention of recurrent VTE is based on the results of the EINSTEIN DVT and EINSTEIN PE trials, and the EINSTEIN EXT and EINSTEIN CHOICE trials, respectively. This review provides an updated overview of these completed EINSTEIN studies in adult patients, including results of subanalyses in patients at high risk of recurrent VTE, and discusses the emerging data from the EINSTEIN Jun...
Source: Blood Coagulation and Fibrinolysis - April 1, 2019 Category: Hematology Tags: REVIEW ARTICLES Source Type: research

Recurrent superficial venous thrombophlebitis because of mutations in the protein C and fibrinogen genes in a young Argentinian female
Hypodysfibrinogenemia and protein C deficiency are coagulopathies and in this report, we describe a young patient with both defects confirmed by molecular genetic tests. The patient was a 24-year-old woman referred for recurrent thrombophlebitis and finally deep venous thrombosis. Routine coagulation studies revealed mild decrease of protein C (0.49 IU, reference values 0.7–1.40 IU) and hypodysfibrinogenemia (0.88 g/l and 1.83 g/l for activity and antigen, respectively, reference values 2.0–4.0 g/l). Direct sequencing analyses were performed on FGA, FGB, and FGG genes to confirm hypodysfibrinogenemia ...
Source: Blood Coagulation and Fibrinolysis - February 8, 2019 Category: Hematology Tags: MUTATION REPORTS Source Type: research

A case of a severe factor XI deficiency in a Chinese woman with heavy menorrhagia
The current study was to elucidate the molecular defect in a 32-year-old Chinese woman with heavy menorrhagia and delayed wound healing. The F11 gene was amplified by PCR and screened for mutations. Then identified mutations were analyzed by in-silico programs and molecular modeling analysis. This woman was found to have severely low levels of factor XI (FXI) (FXI:C: 2.0%; FXI:Ag: 5.4%) by surgical screening. Further DNA sequencing of F11 reveled a novel mutation (p.Ser295Ile) in the Ap4 domain and an already known mutation (p.Trp228stop) in the Ap3 domain. Pedigree analysis showed that the new mutation was inherited from ...
Source: Blood Coagulation and Fibrinolysis - February 8, 2019 Category: Hematology Tags: MUTATION REPORTS Source Type: research

Successful discontinuation of eltrombopag in one child with refractory primary immune thrombocytopenia and literature review
We described a pediatric case of RITP successfully treated with eltrombopag. The platelet count remained normal after 4 years of drug withdrawal. No adverse effect was observed during the treatment. Eltrombopag should be considered well tolerated and effective and has minimal side effects in the treatment of RITP in children, and response can be maintained after eltrombopag is discontinued. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - February 8, 2019 Category: Hematology Tags: CASE REPORTS Source Type: research

Atypical hemolytic–uremic syndrome: recurrent phenotypic expression of a patient with: MCP: gene mutation combined with risk haplotypes
We bring the case of a 38-year-old man who was presented to the emergency department with nausea, fever, and choluria, 4 days after the ingestion of raw oysters. Analytical study revealed thrombocytopenia and acute kidney injury that were associated to a possible thrombotic microangiopathy. Therapeutic plasma exchange was started and resolution of the manifestations was obtained. To identify the cause of the thrombotic microangiopathy a molecular study was performed and a pathogenic variant in the MCP gene, c.287-2A>G (splice acceptor) in heterozygous state with a concomitant presence of both risk haplotypes, MCPggaac a...
Source: Blood Coagulation and Fibrinolysis - February 8, 2019 Category: Hematology Tags: CASE REPORTS Source Type: research

Pseudo-thrombocytopenia after autologous stem cell transplantation
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - February 8, 2019 Category: Hematology Tags: CASE REPORTS Source Type: research

Effects of a protective agent on freeze-dried platelet-rich plasma
In this study, we investigated the effects of a protective agent on freeze-dried platelet-rich plasma (FD-PRP) after a 12-week preservation period. Platelet structure was measured by transmission electron microscopy (TEM), and the expression levels of procaspase activating compound (PAC)-1 and CD62P were measured by flow cytometry. The levels of transforming growth factor-beta (TGF-β), platelet-derived growth factor (PDGF) and vascular endothelial growth factor (VEGF) were determined by ELISA. The effect of FD-PRP on cell proliferation was measured by cell counting. TEM revealed that most platelets were intact, and th...
Source: Blood Coagulation and Fibrinolysis - February 8, 2019 Category: Hematology Tags: ORIGINAL ARTICLE Source Type: research

A history of prophylaxis in haemophilia
Prophylaxis entails long-term continuous intravenous administration of concentrates of the deficient factor with a view to preventing spontaneous bleeds and the development of hemophilic arthropathy. Initiation of prophylaxis at an early age and continuous uninterrupted factor administration in patients with hemophilia have been hailed as essential by such organizations. The most widely used prophylaxis regimens include the Swedish (Malmö), the Dutch and the Canadian protocols. Different international groups have hailed prophylaxis as the most effective treatment in patients with hemophilia. Prophylaxis is effective...
Source: Blood Coagulation and Fibrinolysis - February 8, 2019 Category: Hematology Tags: REVIEW ARTICLE Source Type: research

May restricted specificity of commercially available plasmin inhibitor activity assays affect correct diagnosis of antiplasmin deficiency?
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - January 1, 2019 Category: Hematology Tags: LETTER TO THE EDITOR Source Type: research

Safe childbirth for a type 1 antithrombin-deficient woman with novel mutation in the SERPINC1 gene undergoing antithrombin concentrate therapy
Inherited antithrombin (AT) deficiency is an autosomal dominant thrombotic disorder. We encountered a case of inherited type I AT deficiency and identified the causative mutation; a novel c.7430A>G missense mutation in the SERPINC1 gene in which tyrosine was substituted for cysteine at the 292nd amino acid. A recombinant AT protein with the 7430A>G mutation was not detected in cell lysates or culture supernatants. And then, our patient without personal or family history of thrombosis was pregnant woman with asymptomatic AT deficiency. Our patient treated with only AT concentrate therapy during pregnancy and she was a...
Source: Blood Coagulation and Fibrinolysis - January 1, 2019 Category: Hematology Tags: CASE REPORTS Source Type: research

Probable drug–drug interaction between dabigatran and quinidine resulting in thrombin time rebound despite multiple idarucizumab doses
Dabigatran is a direct thrombin inhibitor that was approved as an alternative to warfarin because it offers the benefit of predictable pharmacokinetic properties, favorable safety profile and ease of administration. Despite the improved safety profile, dabigatran use can lead to bleeding events. The bleeding risk associated with dabigatran is higher in the setting of renal impairment or drug–drug interactions resulting in supratherapeutic serum concentrations. Unfortunately, clinically significant interactions are not always identified by providers, especially in the case of infrequent drug–drug combinations. D...
Source: Blood Coagulation and Fibrinolysis - January 1, 2019 Category: Hematology Tags: CASE REPORTS Source Type: research

Genotype analysis and identification of novel mutations in a multicentre cohort of patients with hereditary factor X deficiency
The objective was to examine the genotypic and phenotypic characteristics of individuals with hereditary factor X deficiency (FXD), a rare autosomal recessive bleeding disorder caused by mutations in the F10 gene located on chromosome 13q34-ter. To date, 149 F10 mutations have been identified as contributory to FXD. Three open-label phase 3 trials enrolled individuals with mild, moderate, or severe FXD. Individuals received plasma-derived factor X concentrate as routine prophylaxis, to treat bleeds, and/or during or after surgery. F10 genotyping was performed (studies 1 and 2) or genotype data was collected at screening (s...
Source: Blood Coagulation and Fibrinolysis - January 1, 2019 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Low-molecular-weight heparin followed by rivaroxaban or not for the prevention of deep venous thromboembolism after total knee arthroplasty
To observe the effect of LMWH followed by rivaroxaban for the prevention of DVT after TKA. Between June 2015 and November 2017 as well as in accordance with the inclusion criteria, 118 patients undergoing TKA were randomly allocated to two groups. Patients in group A were applied LMWH, whereas patients in group B received LMWH followed by rivaroxaban postoperatively. The two groups were foIlowed up and compared for the incidence of DVT by color Doppler ultrasonography scan. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), albumin (ALB), Hb and D-Dimer were also compared. No statistically significant differen...
Source: Blood Coagulation and Fibrinolysis - January 1, 2019 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Inhibitor development in patients with congenital factor VII deficiency, a study on 50 Iranian patients
Congenital factor VII (FVII) deficiency is a rare bleeding disorder with an estimated prevalence of 1 per 500 000 in the general population. On-demand replacement therapy is the main therapeutic choice in patients with congenital FVII deficiency. Inhibitor formation against exogenous FVII is very rare and can cause challenges in the management of the disorder. The present study was conducted to assess the prevalence of FVII inhibitor in 50 patients with congenital FVII deficiency under on-demand or prophylaxis treatment by recombinant activated FVII. All patients with confirmed congenital FVII deficiency were assessed fo...
Source: Blood Coagulation and Fibrinolysis - January 1, 2019 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Relationship between sex hormone binding globulin and blood coagulation in women on postmenopausal hormone treatment
Postmenopausal hormone therapy increases the risk of venous thrombosis. Sex hormone binding globulin (SHBG) is a suggested marker of ‘total estrogenicity’. The study objective was to evaluate the impact of hormone therapy on SHBG and the association with coagulation variables. The study populations comprised 202 healthy postmenopausal women randomized to treatment with low-dose or conventional-dose hormone therapy, tibolone or raloxifene (RET-study) and 140 women with a history of venous thrombosis randomized to conventional-dose hormone therapy or placebo (EVTET-study). SHBG was determined in serum collected a...
Source: Blood Coagulation and Fibrinolysis - January 1, 2019 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Predicting mortality in patients with disseminated intravascular coagulation after cardiopulmonary bypass surgery by utilizing two scoring systems
We evaluated clinical and laboratory biomarkers of disseminated intravascular coagulation (DIC) following cardiac surgery in the cardiothoracic surgical ICU (CTICU) to predict mortality. We retrospectively analyzed CTICU patients with suspected DIC identified from the hospital laboratory database, and calculated International Society on Thrombosis and Haemostasis (ISTH) and the Japanese Association for Acute Medicine (JAAM) DIC scores to predict DIC-related mortality. The predictive accuracy of the JAAM and ISTH DIC scoring system were then assessed by logistic regression analysis and receiver operative characteristics ana...
Source: Blood Coagulation and Fibrinolysis - January 1, 2019 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

What concentration of tranexamic acid is needed to inhibit fibrinolysis? A systematic review of pharmacodynamics studies
Intravenous tranexamic acid (TXA) reduces death because of bleeding in patients with trauma and postpartum haemorrhage. However, in some settings intravenous injection is not feasible. To find different routes of administration, we first need to determine the minimal concentration of TXA in the blood that is required to inhibit fibrinolysis. We conducted a systematic review of in-vitro and in-vivo pharmacodynamics studies. We searched MEDLINE, EMBASE, OviSP, and ISI Web of Science from database inception to November 2017 for all in-vitro (including simulated clotting models) or in-vivo studies reporting the relationship b...
Source: Blood Coagulation and Fibrinolysis - January 1, 2019 Category: Hematology Tags: REVIEW ARTICLE Source Type: research

Protein C deficiency (a novel mutation: ala291Thr) with systemic lupus erythematosus leads to the deep vein thrombosis
The current study aims to explore the phenotype and genotype of a mutation Ala291Thr, which responsible for type I protein C (PC) deficiency in a Chinese woman. The PROC antigen was tested with chromogenic substrate method. PROC gene were amplified by PCR with direct sequencing. Bioinformatics and model analysis were used to study the harm of the mutation. PC activity (PC: A) levels of three members were reduced to 39, 57 and 56%, respectively, PC: antigen was decreased parallelly same as PC: A. Sequencing analysis showed proband with a novel heterozygous c.997G>A point mutation in exon 9 of PROC gene resulting in Ala29...
Source: Blood Coagulation and Fibrinolysis - November 20, 2018 Category: Hematology Tags: MUTATION REPORT Source Type: research

Fatal pulmonary embolism and pulmonary hemorrhage in lupus anticoagulant hypoprothrombinemia syndrome: a case report and review of literature
We report a novel case of LAHS in which the patient experienced the sequence of hemorrhage–thrombosis–hemorrhage before eventually dying of fatal pulmonary embolism and pulmonary hemorrhage. Specifically, she presented with multiple gastrointestinal bleeding episodes, followed by multifocal subdural hematomas, pulmonary embolism after normalization of prothrombin activity levels with immunosuppression, and finally with fatal pulmonary hemorrhage after enoxaparin treatment for pulmonary embolism. This case illustrates the importance of recognizing early minor bleeding episodes, and detecting specific antiprothro...
Source: Blood Coagulation and Fibrinolysis - November 20, 2018 Category: Hematology Tags: CASE REPORTS Source Type: research

Low-dose, short course alteplase treatment of submassive pulmonary embolism: a case series from the National Institutes of Health Clinical Center
Guidelines-recommend thrombolytic therapy for pulmonary embolism in patients with severe hemodynamic compromise and low risk of bleeding. Thrombolytics in submassive pulmonary embolism have an unfavorable risk/benefit ratio and remain controversial. Based on our experience with extensive, lower extremity thrombi, nine patients with symptomatic, submassive pulmonary embolisms (five medical, four surgical) were treated with low-dose alteplase ( (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - November 20, 2018 Category: Hematology Tags: CASE REPORTS Source Type: research

Prevalence of factor VIII inhibitors among Afghan patients with hemophilia A: a first report
Prevalence of inhibitors in Afghan hemophilia patients has not been reported previously. Our aim was to determine the prevalence of factor VIII inhibitors among hemophilia A patients from the Kabul province of Afghanistan to identify and characterize the pattern of inhibitor formation. Clinical information and blood samples were collected from three hemophilia centers in Kabul, Afghanistan. Plasma samples were obtained from 62 patients with severe (80.5%) and 15 patients with moderate hemophilia A (19.5%) in this cross-sectional study design. All the patients were receiving on-demand treatment. The Nijmegen modification of...
Source: Blood Coagulation and Fibrinolysis - November 20, 2018 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Molecular genetic diagnosis of Tunisian Glanzmann thrombasthenia patients reveals a common nonsense mutation in the ITGA2B gene that seems to be specific for the studied population
This study was aimed at identifying Glanzmann thrombasthenia-associated novel mutations in Tunisian patients. Seven unrelated Glanzmann thrombasthenia patients issued from high consanguineous families (86%; 6/7 of the patients) were studied. Glanzmann thrombasthenia diagnoses were based on patients’ bleeding histories and platelet aggregation tests. Screening of ITGA2B and ITGB3 genes was performed by denaturing high-performance liquid chromatography (DHPLC) analysis. Amplicons with abnormal elution profiles were subjected to direct sequencing. DHPLC/sequencing analysis identified a pathogenic homozygous mutation in ...
Source: Blood Coagulation and Fibrinolysis - November 20, 2018 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Prediction of bleeding by thromboelastography in ICU patients with haematological malignancy and severe sepsis
ICU patients with haematological malignancy have an increased risk of bleeding. Recently, global haemostatic methods such as thromboelastography (TEG) have gained impact in evaluating coagulation. The aim of this study was to observe whether TEG could predict bleeding in haematological ICU patients with severe sepsis. Post-hoc single-centre analysis of patients with haematological malignancy included in the Scandinavian Starch for Severe Sepsis/Septic Shock (6S) trial. Clinical characteristics, TEG measurements and details regarding bleeding complications were retrieved from the 6S database. The association between TEG and...
Source: Blood Coagulation and Fibrinolysis - November 20, 2018 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Post-transcriptional, post-translational and pharmacological regulation of tissue factor pathway inhibitor
Tissue factor (TF) pathway inhibitor (TFPI) is an endogenous natural anticoagulant that readily inhibits the extrinsic coagulation initiation complex (TF-FVIIa-Xa) and prothrombinase (FXa, FVa and calcium ions). Alternatively, spliced TFPI isoforms (α, β and δ) are expressed by vascular and extravascular cells and regulate thrombosis and haemostasis, as well as cell signalling functions of TF complexes via protease-activated receptors (PARs). Proteolysis of TFPI plays an important role in regulating physiological roles of the TF pathway in host defense and possibly haemostasis. Elimination of TFPI inhibiti...
Source: Blood Coagulation and Fibrinolysis - November 20, 2018 Category: Hematology Tags: REVIEW ARTICLES Source Type: research

Sex, gender and venous thromboembolism: do we care enough?
The role of sex and gender in determining clinical presentation, diagnostic approach and outcomes of venous thromboembolism is not fully and systematically addressed, except for hormone-related events in women. A lack of knowledge is also apparent regarding drug prescription patterns, physician bias, enrolment in clinical studies and analysis of sex-related confounders in preclinical and clinical studies. As was shown for cardiovascular disease, ignoring sex and gender in medicine can have important impact on outcomes, including mortality. In this review, we seek to address some aspects of venous thromboembolism such as ep...
Source: Blood Coagulation and Fibrinolysis - November 20, 2018 Category: Hematology Tags: REVIEW ARTICLES Source Type: research

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