Abstract：
:The development of antibodies against infused factor VIII (FVIII) in patients with haemophilia A is a serious complication leading to poorly controlled bleeding and increased morbidity. No treatment has been proven to reduce high titre antibodies in patients who fail immune tolerance induction or are not candidates for it. The Rituximab for the Treatment of Inhibitors in Congenital Hemophilia A (RICH) study was a phase II trial to assess whether rituximab can reduce anamnestic FVIII antibody (inhibitor) titres. Male subjects with severe congenital haemophilia A and an inhibitor titre ≥5 Bethesda Units/ml (BU) following a FVIII challenge infusion received rituximab 375 mg/m² weekly for weeks 1 through 4. Post-rituximab inhibitor titres were measured monthly from week 6 through week 22 to assess treatment response. Of 16 subjects who received at least one dose of rituximab, three (18.8%) met the criteria for a major response, defined as a fall in inhibitor titre to <5 BU, persisting after FVIII re-challenge. One subject had a minor response, defined as a fall in inhibitor titre to <5 BU, increasing to 5-10 BU after FVIII re-challenge, but <50% of the original peak inhibitor titre. Rituximab is useful in lowering inhibitor levels in patients, but its effect as a solo treatment strategy is modest. Future studies are indicated to determine the role of rituximab as an adjunctive therapy in immune tolerisation strategies.
journal_name
Thromb Haemostjournal_title
Thrombosis and haemostasisauthors
Leissinger C,Josephson CD,Granger S,Konkle BA,Kruse-Jarres R,Ragni MV,Journeycake JM,Valentino L,Key NS,Gill JC,McCrae KR,Neufeld EJ,Manno C,Raffini L,Saxena K,Torres M,Marder V,Bennett CM,Assmann SFdoi
10.1160/TH14-01-0078subject
Has Abstractpub_date
2014-09-02 00:00:00pages
445-58issue
3eissn
0340-6245issn
2567-689Xpii
14-01-0078journal_volume
112pub_type
杂志文章,多中心研究abstract：:Hereditary defects of the von Willebrand factor (VWF) gene cause von Willebrand's disease (VWD) which shows great variability dependent on the nature and location of the mutation. We here describe the characteristics of a substitution of methionine for threonine 1156 in the D3 domain of the VWF, i.e. the domain involv...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2002-09-01 00:00:00
abstract：:A series of aromatic amidino compounds were investigated for their inhibitory effect on platelet agglutination and platelet aggregation. Agglutination of fresh or fixed platelets was produced by bovine plasma or by human plasma in combination with ristocetin, while aggregation of fresh platelets was induced by ADP, th...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1978-04-30 00:00:00
abstract：:Given conflicting data on temporal variability in pharmacodynamic platelet responses to clopidogrel, we investigated platelet reactivity on clopidogrel and aspirin for up to six months after elective percutaneous coronary intervention (PCI) with drug-eluting stents. Platelet reactivity was determined in 102 patients b...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章
doi：10.1160/TH15-03-0257
更新日期：2015-11-01 00:00:00
abstract：:AZD0837 is an investigational oral anticoagulant which is converted to the active form, AR-H067637, a selective direct thrombin inhibitor. The present study, a multicentre, randomised, parallel-group, dose-guiding study, assessed the safety and tolerability of an immediate-release formulation of AZD0837 compared with ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,多中心研究,随机对照试验
doi：10.1160/TH09-07-0509
更新日期：2010-03-01 00:00:00
abstract：:Although associated with adverse outcomes in other cardiopulmonary diseases, limited evidence exists on the prognostic value of anaemia in patients with acute pulmonary embolism (PE). We sought to examine the associations between anaemia and mortality and length of hospital stay in patients with PE. We evaluated 14,27...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,多中心研究
doi：10.1160/TH11-04-0208
更新日期：2011-08-01 00:00:00
abstract：:Fibrin sealant use in the United States has been limited because of the lack of a commercially available, government approved product. However, the significant need and usefulness of this material at the time of surgical operations has stimulated alternative sources of production. At the University of Virginia, the Bl...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：1995-07-01 00:00:00
abstract：:Antiphospholipid antibodies (aPL) have been associated with thrombosis and pregnancy losses in patients diagnosed with antiphospholipid syndrome (APS) and enhance thrombus formation in vivo in mice, but the mechanism of thrombosis by aPL is not completely understood. It has been proposed that aPL may affect endothelia...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2000-09-01 00:00:00
abstract：:The effects on the haemostatic mechanism of oestrogen therapy, given to prevent bone loss in post-menopausal women, have been investigated. Oestriol succinate was given orally to 10 women at a level of 2 mg/day for 1 month and for a further 3 months with incremental increase of 2 mg each month. 6 of the 10 women were ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1976-04-30 00:00:00
abstract：:Congenital amegakaryocytic thrombocytopaenia (CAMT) is a disorder caused by c-MPL mutations that impair thrombopoietin (TPO) signalling, resulting in a near absence of megakaryocytes (MKs). While this phenotype is consistent in adults, neonates with CAMT can present with severe thrombocytopaenia despite normal MK numb...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH17-06-0433
更新日期：2017-12-01 00:00:00
abstract：:One hundred patients were included in a randomized open trial to assess the systemic factor Xa (FXa) and thrombin inhibitory effect as well as the safety profile of low molecular weight heparin (LMWH) given subcutaneously in conjunction with streptokinase (SK) in patients with acute myocardial infarction (MI). The tre...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章,随机对照试验
doi：
更新日期：1997-01-01 00:00:00
abstract：:The cofactor tetrahydrobiopterin (BH4) is required for nitric oxide (NO) production by all nitric oxide synthase (NOS) enzymes and is a key regulator of cellular redox signalling. When BH4 levels become limiting NOS enzymes become 'uncoupled' and produce superoxide rather than NO. Endothelial cell BH4 is required for ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：10.1160/TH12-06-0424
更新日期：2012-11-01 00:00:00
abstract：:The generation of D-dimer was studied in the course of local thrombolytic therapy of peripheral arterial occlusions with low doses of recombinant human tissue-type plasminogen activator (rt-PA) in 7 patients. Intermittent local application of rt-PA resulted in a marked increase in D-dimer exceeding values usually seen...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1990-10-22 00:00:00
abstract：:The effect of aspirin on LPS-incubation of whole blood was investigated. Aspirin induced a concentration dependent increase (2.5-5-fold at 5 mM aspirin) in LPS-induced appearance of TNF-alpha and fibrinopeptide A (FPA) in plasma, despite the concomitant increase in the inhibitory cytokine IL-100. Aspirin substantially...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2000-06-01 00:00:00
abstract：:Causative mutations in the factor VIII gene of seven unrelated patients with severe haemophilia A were identified using the mutation screening procedure conformation sensitive gel electrophoresis (1) and characterised by direct sequencing. Female family members of all patients had requested either carrier status deter...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1998-04-01 00:00:00
abstract：:Developmental haemostasis is a concept, now universally accepted, introduced by Andrew et al. in the late 1980's. However, coagulation analysers and reagents have changed significantly over the past 15 years. Coagulation testing is known to be sensitive to changes in individual reagents and analysers. We hypothesised ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH05-01-0047
更新日期：2006-02-01 00:00:00
abstract：:Stress may counteract responses to antiplatelet drug treatment. We investigated if adding clopidogrel to aspirin treatment could attenutate stress-induced platelet activation and myocardial ischemia in patients with coronary artery disease (CAD). Thirty-one male patients with documented CAD-treated with aspirin (75-16...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,随机对照试验
doi：10.1160/th07-05-0323
更新日期：2007-12-01 00:00:00
abstract：:An assay of human antiplasmins has been developed utilizing radial diffusion of plasma from wells cut in plasmin-enriched, fibrinogen-agarose plates. After diffusion the fibrinogen is clotted. Zones of fibrin protected from background fibrinolysis develop as the result of plasma antiplasmin activity. A pooled plasma s...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1978-04-30 00:00:00
abstract：:The structures of bovine and human vWF were compared by proteolysis with Staphylococcus aureus V8 protease and rattlesnake venom Protease I. Fragments were analyzed for chain composition, heparin binding, collagen binding, platelet agglutinating activity and recognition by a panel of monoclonal antibodies which reacte...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1990-06-28 00:00:00
abstract：:Site directed mutagenesis was used to construct a t-PA variant that contains an additional glycosylation site in the first kringle domain (T103N) combined with a tetra-alanine substitution in the protease domain (KHRR 296-299 AAAA). This combination variant has a plasma clearance rate that is 4.5-fold slower in rats a...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1993-08-02 00:00:00
abstract：:Normal adults have very few circulating endothelial cells (CECs) in their blood, but increased levels have been shown in association with conditions associated with endothelial damage such as myocardial infarction and stroke. As atrial fibrillation (AF) is associated with a hypercoagulable state and abnormalities of p...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH05-02-0093
更新日期：2005-10-01 00:00:00
abstract：:Factor Xa (fXa)-inhibitors are as effective and safer than vitamin-K-antagonists (VKA) in the treatment of venous thromboembolism (VTE). We previously classified the severity of clinical presentation and course of all major bleeding events from the EINSTEIN, AMPLIFY and HOKUSAI-VTE trials separately. The current aim w...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH16-12-0946
更新日期：2017-10-01 00:00:00
abstract：:Fucoïdans catalyse thrombin inhibition by antithrombin (AT) and heparin cofactor II (HCII); their affinity for each serpin varies according to the seaweed species from which they are extracted, as well as their chemical composition and molecular weight. We extracted a homogeneous fucoïdan fraction from Ascophyllum nod...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1995-11-01 00:00:00
abstract：:Increased red blood cell distribution width (RDW), which is a marker of anisocytosis, is associated with mortality and cardiovascular events in the general population and in patients with heart failure or coronary heart disease. We investigated whether RDW in acute cerebral infarction is predictive of functional outco...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH12-03-0165
更新日期：2012-08-01 00:00:00
abstract：:The Pulmonary Embolism Severity Index (PESI) is a validated clinical prognostic model for patients with acute pulmonary embolism (PE). Our goal was to assess the PESI's inter-rater reliability in patients diagnosed with PE. We prospectively identified consecutive patients diagnosed with PE in the emergency department ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH10-07-0426
更新日期：2010-12-01 00:00:00
abstract：:Although intravital microscopy models of thrombosis in mice have contributed to dissect the mechanisms of thrombus formation and stability, they have not been well adapted to study long-term evolution of occlusive thrombi. Here, we assessed the suitability of the dorsal skinfold chamber (DSC) for the study of thrombol...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH11-10-0705
更新日期：2012-05-01 00:00:00
abstract：BACKGROUND: This study assessed changes in anticoagulation therapy over time in patients with atrial fibrillation (AF). METHODS: Analyses were performed on a claims-based dataset of 4 million health-insured individuals. The study population consisted of patients newly initiating a non-vitamin-K oral anticoagulants (NO...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1055/s-0039-1683428
更新日期：2019-06-01 00:00:00
abstract：:Amniotic fluid inhibited the fibrinolytic, amidolytic and esterolytic activity of urokinase. Kinetic studies with AGLMe demonstrated non-competitive inhibition. A major part of the inhibitory activity could be separated from alpha 1-antitrypsin by ion-exchange chromatography on DEAE-Sephadex A-50. Plasminogen activato...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1980-08-29 00:00:00
abstract：:Tissue factor is a cellular receptor that initiates blood coagulation. The TF gene is constitutively expressed in some extravascular cell types and is inducibly expressed in several vascular cell types, including monocytes, vascular endothelial cells and vascular smooth muscle cells. Functional studies indicate that S...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：1997-07-01 00:00:00
abstract：:The content of free-catecholamines in blood platelets is much higher than in plasma and platelet catecholamines must be taken up from plasma, since platelets lack the enzymes for catecholamine synthesis. There is some evidence that platelet catecholamine content under certain circumstances may be an integrated measure...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1988-10-31 00:00:00
abstract：:Vitamin K-dependent proteins are currently purified from pooled human plasma or produced in mammalian cell culture systems by recombinant DNA technology. Transgenic animals may provide an additional expression system for the production of these therapeutic proteins. Mice have been used to test hybrid genes which direc...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：1997-07-01 00:00:00
