Risk factors for antibody formation in children with hemophilia: methodological aspects and clinical characteristics of the HEMFIL cohort study
Up to 35% of patients with hemophilia A and 5% with hemophilia B develop neutralizing antibodies which can inhibit the therapeutic activity of factor replacement (inhibitors). Despite the clinical relevance of antifactor VIII and IX neutralizing antibodies, there is still a major gap on the knowledge of risk factors for their development. Furthermore, most of the studies on risk factors for inhibitor development come from Caucasian and Afro-American populations. The HEMFIL is a Brazilian prospective cohort study of previously untreated children with hemophilia, which primary aim is to identify new risk factors related to i...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Apoptosis in platelets from adult patients with chronic idiopathic thrombocytopenic purpura
Adult chronic idiopathic thrombocytopenic purpura (cITP) is a chronic and usually life-long haemorrhagic disorder in which enhanced platelet destruction and weakened platelet production lead to thrombocytopenia. Platelets were isolated from blood samples collected from 40 adult patients with cITP and 40 healthy volunteers. Mitochondrial membrane potential (ΔΨm) and plasma membrane phosphatidylserine externalization were determined by flow cytometry, and activation of caspase-3 and expressions of Bax, Bak and Bcl-xL were analysed by western blotting. Flow cytometry showed increased mitochondrial depolarization and lower Î...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Immune thrombocytopenia: options and new perspectives
Immune thrombocytopenia is a haematological, autoimmune disorder characterized by elevated platelet demolition due to the presence of antiplatelet autoantibodies derived from B cells and to an irregular, deficient process of platelets production in bone marrow. In this review, after a brief presentation of ‘old’ strategies used nowadays yet, we focused on new drugs used in the treatment of immune thrombocytopenia and their mechanism of action and posology, basing on the last scientific literature. The observation that CoViD-19 can be associated with immune thrombocytopenia is also put in evidence. Particular attention ...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: REVIEW ARTICLE Source Type: research
Identification of a recurrent missense mutation in the FGA gene likely causing a congenital fibrinogen disorder
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: LETTERS TO THE EDITOR Source Type: research
Subcutaneous injection of mRNA vaccines against severe acute respiratory syndrome coronavirus 2: an option for severe bleeding disorders or anticoagulated patients?
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: LETTERS TO THE EDITOR Source Type: research
Spinal epidural hematoma in a child with hemophilia A with high titer inhibitors and follow-up with prophylactic emicizumab: case report and literature review
Hemorrhage in the central nervous system is the most severe and debilitating manifestation affecting patients with hemophilia A. The spinal epidural space is the most unusual and clinically challenging site of central nervous system hemorrhage in hemophilia A. These patients often show insidious neurological signs and symptoms that delay diagnosis and treatment. We share our experience treating a 4-year-old male patient with severe hemophilia A and high titer inhibitors with a spontaneous spinal epidural hematoma. The patient presented initially with intense headache and neck pain. After blood tests and imaging studies, by...
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: CASE REPORTS Source Type: research
Leukocyte-platelet rich fibrin on the treatment of a large paradental cyst: a novel regenerative approach
Leukocyte-platelet rich fibrin (PRF) is an autologous biomaterial formed by platelets, cytokines, growth factors and cells imprisoned on a fibrin mesh, produced according to Choukroun's protocol. The aim of the present article was to report the use of PRF, associated with a bone substitute, on the regenerative treatment of a large bone defect resulting from the enucleation of a paradental cyst involving the posterior mandible. The treatment resulted in the maintenance of the bone volume, and radiographic evaluation showed new bone formation after 40 days, suggesting an osteogenic and osteoinductive effect. Also, the curren...
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: CASE REPORTS Source Type: research
Genetic analysis of a pedigree with hereditary coagulation factor XII deficiency
The aim of this study was to elucidate the molecular defects of a Chinese family with hereditary coagulation factor XII (FXII) deficiency. The FXII activity (FXII:C) and FXII antigen (FXII:Ag) levels were measured by clotting assay and ELISA, respectively. To identify mutations, the F12 gene sequencing was carried out. ClustalX-2.1-win and four online bioinformatics tools were applied to study the conservatism and harm of the mutation. The proband's FXII:C and FXII:Ag were 3 and 4%, respectively. Sequencing analysis revealed compound heterozygous mutations, including the deletion mutation (c.130delG) resulting in p.E26Sfsâ...
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: MUTATION REPORTS Source Type: research
A novel mutation (Leu60Pro) in a Chinese pedigree with hereditary factor XI deficiency
To analyse F11 gene mutations in a Chinese pedigree with hereditary factor XI (FXI) deficiency and investigate the molecular mechanism. The plasma FXI activity and FXI antigen of the proband and the family members were detected by clotting assay and ELISA, respectively. The F11 gene was amplified by PCR and sequenced directly. Online bioinformatics software were needed to analyse the mutations. The proband showed a prolonged activated partial thromboplastin time (93.3 s), whose FXI activity and FXI antigen were low to 2, 4.5%, respectively. A novel mutation c.233T>C (p.Leu60Pro) in exon 4 and a previously described mutat...
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: MUTATION REPORTS Source Type: research
Maternal thrombin generation and D-dimer levels in obesity and pregnancy: results from the maternal thrombin generation in obesity and pregnancy (MaTOPs) study
Venous thromboembolic disease (VTE) risk increases five-fold antenatally and 14-fold during the puerperium. Obesity significantly increases this risk. The D-dimer assay and more novel Calibrated Automated Thrombogram (CAT) assay laboratory tests display potential for use in VTE risk stratification in pregnancy, although to date, research in the performance characteristics of these tests in obese and nonobese pregnant populations is limited.
The aim of this study was to compare D-dimer and thrombin generation levels in obese and nonobese pregnant women.
Pregnant women were recruited and categorised, as obese (BMI ≥3...
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Clinical characteristics and outcomes of patients with isolated pulmonary embolism
The clinical relevance of concomitant deep vein thrombosis (DVT) in patients with pulmonary embolism remains controversial. The aim of the present study was to assess clinical characteristics of patients with isolated pulmonary embolism, thereby investigating isolated pulmonary embolism related clinical factors.
Patients hospitalized for pulmonary embolism who underwent DVT workup within 3 days of pulmonary embolism diagnosis were retrospectively classified into two groups: patients with isolated pulmonary embolism and patients with DVT-associated pulmonary embolism (DVT-PE). The clinical, laboratorial and radiological ...
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Homozygous methylentetrahydrofolate reductase C667T genotype anticipates age at venous thromboembolism by one decade
The aim of the study was to compare age at first venous thromboembolism (VTE), plasma homocysteine and activated partial thromboplastin time ratio (aPTTr) amongst unprovoked VTE patients with the methylentetrahydrofolate reductase (MTHFR) C667T genotypes, and to identify predictors of age at first VTE, of plasma homocysteine and of the aPTTr; to evaluate whether heterozygous or homozygous prothrombin (PT) G20210A mutation lowered the age at first VTE when associated with MTHFR TT. Retrospective cohort study on 259 MTHFR TT, 76 MTHFR TC and 64 MTHFR CC participants with unprovoked VTE; each participant contributed age, sex,...
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Risk of venous thromboembolism and endocrine therapy in older women with breast cancer in the United States
Our aim was to evaluate the risk of venous thromboembolism (VTE) with tamoxifen and aromatase inhibitor in older women with breast cancer in the United States. The SEER-Medicare-linked database (2007--2013) was used for women of at least 65 years of age diagnosed with breast cancer in the United States. Logistic regression was used to examine unadjusted and adjusted odds ratios (OR) with 95% confidence intervals (CIs) for the risk of VTE. There were 178 059 women aged at least 65 years with breast cancer in the United States. Twenty-two thousand and forty-two (12.4%) women received tamoxifen, 64 384 (36.2%) women r...
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Management of surgery, menorrhagia and child-birth for patients with unclassified bleeding disorders: a systematic review of cohort studies
Unclassified bleeding disorders account for 2.6% of all new bleeding disorder registrations in the UK. The management of the bleeding phenotype associated with these disorders is poorly described. Systematic review and meta-analysis to determine the bleeding rates associated with tranexamic acid, desmopressin, platelet transfusion, plasma transfusion and recombinant activated factor VII, for patients with unclassified bleeding disorders undergoing surgery, childbirth or with menorrhagia. We searched for randomized controlled trials in MEDLINE, Embase, The Cochrane Central Register of Controlled Trials, PubMed, ISI Web of S...
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Viscoelastic testing to assess the effects of rapid fibrinogen concentrate administration after cardiopulmonary bypass: insights from the REPLACE study
Haemorrhage during and following surgery results in increased morbidity and mortality. Low plasma fibrinogen levels have been associated with increased blood loss and transfusion requirements. Fibrinogen supplementation has been shown to reduce bleeding in coagulopathic patients. This post hoc study evaluated fibrinogen repletion and pharmacokinetic data from the REPLACE study. One hundred and fifty-two adult patients undergoing elective aortic surgery requiring cardiopulmonary bypass (CPB) with defined bleeding of 60–250 g at first 5 min bleeding mass were included in the phase III trial. Patients were randomized to...
Source: Blood Coagulation and Fibrinolysis - August 21, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
