Excluding JAK2 V617F mutation analysis from the primary immune thrombocytopenia ‘diagnosis of exclusion’
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - November 26, 2022 Category: Hematology Tags: LETTERS TO THE EDITOR Source Type: research

The role of immune status, viral co-infections and paraproteinemia in development of venous thromboembolism in HIV
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - November 26, 2022 Category: Hematology Tags: LETTERS TO THE EDITOR Source Type: research

Phenotypic and genotypic characterization of two factor VII deficiency patients from southeastern China
The congenital factor VII deficiency (FVIID) is a rare autosomal recessive haemorrhagic disease caused by mutations in the F7 gene. The aim of this study was to identify the mutations causing FVII deficiency and explain the genotype–phenotype association in two unrelated Chinese patients. Mutation detection was conducted by sequencing the whole F7 gene coding exons, exon–intron boundaries and the untranslated regions of 3′ and 5′. Then, the genetic information was analyzed to predict the structures of the mutated proteins. A total of four different mutations were detected, including three missense mutations (c.64G>...
Source: Blood Coagulation and Fibrinolysis - November 26, 2022 Category: Hematology Tags: MUTATION REPORT Source Type: research

Phenotypic variation in severe hemophilia A is related to endogenous thrombin potential and plasma levels of factor VII
This study aimed to evaluate the association of biomarkers and coagulation parameters to the differential hemorrhagic profile of severe hemophilia A patients. Polymorphisms in the genes of proteins C and S, factors V and VII and prothrombin were evaluated in a group of severe hemophilia A patients with a broad spectrum of bleeding profile. Plasma levels of coagulation factors and thrombin generation were also analyzed. This study included 59 Brazilian hemophilia A patients who were allocated into low bleeding profile (LBP; n = 33) and high bleeding profile (HBP; n = 26) groups based on their joint and muscle bleedi...
Source: Blood Coagulation and Fibrinolysis - November 26, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Anticoagulant and antiplatelet activity of aqueous extracts of Citrus sinensis and Lippia alba: interactions and potential for the development of antithrombotics
Citrus sinensis and Lippia alba are herbal medicines widely used in the form of tea (infusion, decoction), which ethanolic extracts have already shown great anticoagulant activity in vitro. For this reason, they seem to be excellent candidates for the development of new antithrombotics and also have the potential to interact with them. The aim of this study was to evaluate the activity of aqueous extracts in blood coagulation and platelet aggregation, in addition to analysing the micromolecular composition of these species. Thrombin generation test (TGT) by the Calibrated Automated Thrombogram method and Platelet Agg...
Source: Blood Coagulation and Fibrinolysis - November 26, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Venous thromboembolism in pediatric inflammatory bowel disease: an 11-year population-based nested case–control study in Canada
To investigate the occurrence of venous thromboembolism (VTE), clinical characteristics, risk factors, treatment outcomes, and anticoagulation safety in pediatric inflammatory bowel disease (IBD) over an 11-year period. A nested case–control study within an identified cohort was performed amongst children with IBD from 2009 to 2020 in Canada. There were 890 hospitalizations during the study period, and 15 venous thromboembolic events (1.69%) among 12 patients occurred, including 12 with ulcerative colitis and three with Crohn's disease. VTE proportions were significantly different between female (2.7%) and male (0.8%) pa...
Source: Blood Coagulation and Fibrinolysis - November 26, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Tissue factor-dependent coagulation activation in intracranial neoplasms: a comparative study
The aim of the study was to investigate the concentration and activity of tissue factor (TF) and Tissue factor pathway inhibitor (TFPI) as well as the concentration of thrombin–antithrombin (TAT) complexes in patients with primary and metastatic intracranial neoplasms. The study included 69 patients with an average age of 62 years. Twenty-one patients were diagnosed with gliomas, 18 meningioma stage II (M) patients, and 30 metastatic brain tumour cases (Meta). The control group consisted of 30 individuals with a mean age of 57 years. In the plasma of all the participants and in tumour tissue-derived homogenate, the c...
Source: Blood Coagulation and Fibrinolysis - November 26, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

The effect of tranexamic acid on myocardial injury in cardiac surgical patients: a systematic review and meta-analysis
Tranexamic acid (TXA) is one of the most commonly used antifibrinolytic agents for surgical patients. However, the effect of TXA on myocardial injury remains controversial. We systemically reviewed literature regarding the effectiveness of TXA on myocardial injury in patients who have undergone a cardiac surgery. We included all randomized controlled trials (RCTs) comparing TXA and control (saline) in cardiac surgical patients. Relevant studies were identified by a comprehensive electronic literature search from database inception to 15 August 2021. A standardized data extraction form was used to collect methodological and...
Source: Blood Coagulation and Fibrinolysis - November 26, 2022 Category: Hematology Tags: REVIEW ARTICLE Source Type: research

Reversal of life-threatening bleeding with protamine sulfate in a patient with plasma cell leukemia
Plasma cell disorders, such as multiple myeloma, can cause numerous derangements of hemostasis. In this case report, we present a life-threatening coagulopathy in a patient with progressing multiple myeloma in which the antibody-producing heparin-like activity is a free light chain. The patient's bleeding was successfully treated using protamine sulfate, which then allowed treatment of her plasma cell leukemia. In the literature, other authors have reported similar patients who have responded to protamine sulphate either in vitro or in vivo, providing further evidence for the role of protamine sulfate in the reversal of co...
Source: Blood Coagulation and Fibrinolysis - October 1, 2022 Category: Hematology Tags: CASE REPORTS Source Type: research

Presentation of concurrent thrombotic thrombocytopenic purpura and Graves’ disease
We present a case of acquired TTP in the setting of thyrotoxicosis from Graves’ disease. Our patient improved with standard treatment of both TTP and thyrotoxicosis. A retrospective review of patients with TTP at our institution demonstrated that 32% had another autoimmune disorder, highlighting the concept of polyautoimmunity. These findings suggest an association between TTP and uncontrolled autoimmune disease. In patients with newly diagnosed TTP, physicians should evaluate for other autoimmune diseases and check thyroid function tests. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 1, 2022 Category: Hematology Tags: CASE REPORTS Source Type: research

Hemophilia in the south of Tunisia
Hemophilia is a rare constitutional hemorrhagic disorder. There is insufficient epidemiological data on hemophilia in Tunisia. To describe the epidemiological, clinical, therapeutic, and outcome of a cohort of patients with hemophilia in southern Tunisia. A retrospective study was conducted on patients with hemophilia at the Hemophilia Treatment Center of Southern Tunisia in Sfax over 38 years (from January 1982 to December 2020). Data were collected in a regional hemophilia registry of the South Tunisian center. We collected 141 cases of hemophilia, 85% of whom had hemophilia A and 15% had hemophilia B. The severe form re...
Source: Blood Coagulation and Fibrinolysis - October 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Hemorrhagic complications in individuals with liver disease submitted to minor oral surgery
Individuals with chronic liver disease (CLD) have an increased risk of bleeding from thrombocytopenia and changes in hemostasis. The aim of this study was to evaluate the frequency of and the factors associated with the occurrence of bleeding in CLD patients who underwent dental surgical procedures. This was a retrospective study whose data were collected in a hospital dentistry service between 2010 and 2016. The patients were referred from the gastroenterology and liver transplantation services of a university hospital for dental treatment. The study followed the STROBE guidelines. Among the 71 surgical procedures perform...
Source: Blood Coagulation and Fibrinolysis - October 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Harmonizing factor assay-related testing performed in a large laboratory network
Coagulation factor testing is commonly performed within haemostasis laboratories, either to assess for bleeding disorders, such as haemophilia, or to investigate unexplained prolongation in routine coagulation assays. The aim of this evaluation was to harmonize procedures and normal reference ranges (NRRs) for investigation of coagulation factors on the ACL TOP 50 family of instruments in a large laboratory network. We employed comparative evaluations using newly installed ACL TOPs 550 and 750 and HemosIL reagents vs. existing ‘reference’ instrumentation and reagents, predominantly Stago and Siemens, as well as assessm...
Source: Blood Coagulation and Fibrinolysis - October 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Comparing the efficacy and safety of direct oral anticoagulants versus Vitamin K antagonists in patients with antiphospholipid syndrome: a systematic review and meta-analysis
Thromboprophylaxis is the cornerstone strategy for thrombotic antiphospholipid syndrome (APS). Data comparing direct oral anticoagulants (DOACs) to Vitamin K antagonists (VKAs) in the secondary prevention of thrombosis in APS patients remain contentious. We aim to review and analyse literature on the efficacy and safety of DOACs compared with VKAs in treating patients with APS. A literature search was performed from inception to 31 December 2021. Subgroups were analysed based on the risk stratification of APS profiles and different DOAC types. A total of nine studies with 1131 patients were included in the meta-analy...
Source: Blood Coagulation and Fibrinolysis - October 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research

Evaluation of primary care physicians’ approaches to hemophilia and bleeding disorders: a questionnaire survey
This study aimed to determine and compare the approach of primary care physicians (including general practitioners) and emergency physicians with a questionnaire including simulation-based cases on hemophilia. This simulation and two-stage questionnaire study was conducted with 244 participants. Before–after questionnaires, two case simulations, a brief presentation, and statistical analysis were performed. Participants mostly preferred tests, such as prothrombin time (PT) or partial thromboplastin time (PTT) to bleeding time for primary hemostasis (PT/PTT n: 192, 84.2%, bleeding time n: 94, 41.2%). Similar results were ...
Source: Blood Coagulation and Fibrinolysis - October 1, 2022 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research