Inhibitor development in hemophiliacs: the roles of genetic versus environmental factors.


:Approximately 5 to 7% of patients with hemophilia A have inhibitory antibodies to factor (F) VIII, which increases to approximately 13% in patients with severe disease. The strongest determinant of the risk of inhibitor development identified is the type of mutation in the FVIII gene that gives rise to the disease. However, accumulating evidence clearly indicates that other genetic factors (e.g., major histocompatibility complex alleles and other immune-modulatory genes) and factors associated with treatment (e.g., type of FVIII concentrate, route of administration, and age of first exposure) may also influence the risk of inhibitor development. There is much interest in identifying such genetic and treatment-related factors to help minimize the risk of inhibitor development and improve treatment outcomes.


Semin Thromb Hemost


Lee CA,Lillicrap D,Astermark J




Has Abstract


2006-06-01 00:00:00








32 Suppl 2


  • Inhibition of platelet function: does it offer a chance of better cancer progression control?

    abstract::Thrombocytosis is frequently (10 to 57%) observed in cancer patients. Although the mechanisms underlying thrombocytosis are not yet fully elucidated, tumor-derived factors with thrombopoietin-like activity, growth factors, platelet-derived microparticles, and factors released from bone marrow endothelial cells as well...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Sierko E,Wojtukiewicz MZ

    更新日期:2007-10-01 00:00:00

  • Thrombolytic activity of defibrotide against old venous thrombi.

    abstract::The age of the thrombus is probably a very important determinant of the outcome of thrombolysis. The clinical potential for rapidly dissolving thrombi by thrombolytic therapy is considerable because restoration of the blood flow can rescue the jeopardized district served by the occluded vessel such as for myocardial i...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Niada R,Porta R,Pescador R,Mantovani M,Prino G

    更新日期:1989-10-01 00:00:00

  • Assays of von Willebrand factor-cleaving protease: a test for diagnosis of familial and acquired thrombotic thrombocytopenic purpura.

    abstract::Endothelial cells secrete von Willebrand factor (vWF) multimers that are larger than those found in the circulating plasma. These very large multimeric forms of vWF, capable of spontaneously binding to and agglutinating the blood platelets under conditions of high fluid shear rate, are degraded by a specific metallopr...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Furlan M,Lämmle B

    更新日期:2002-04-01 00:00:00

  • Thromboprophylactic effect of low molecular weight heparin started in the evening before elective general abdominal surgery: a comparison with low-dose heparin.

    abstract::A prospective randomized double-blind trial was performed comparing conventional low-dose heparin with a LMWH fragment (Kabi 2165, Fragmin) for thromboprophylaxis in elective general abdominal surgical patients. The first dose of the fragment was given in the evening before surgery, and thereafter every evening. There...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验


    authors: Bergqvist D,Burmark US,Frisell J,Guilbaud O,Hallböök T,Horn A,Lindhagen A,Ljungner H,Ljungström KG,Mätzsch T

    更新日期:1990-10-01 00:00:00

  • An association between maternal smoking and preeclampsia in Japanese women.

    abstract::In order to determine whether maternal smoking before or during pregnancy, or both, is associated with a reduced risk for preeclampsia in Japanese subjects, we conducted a case-control study that took other risk factors for preeclampsia into consideration. Seventy-one preeclampsia patients were matched with 142 contro...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Kobashi G,Ohta K,Hata A,Shido K,Yamada H,Fujimoto S,Kondo K

    更新日期:2002-12-01 00:00:00

  • Newly-Recognized Roles of Factor XIII in Thrombosis.

    abstract::Arterial and venous thromboses are major contributors to coagulation-associated morbidity and mortality. Greater understanding of mechanisms leading to thrombus formation and stability is expected to lead to improved treatment strategies. Factor XIII (FXIII) is a transglutaminase found in plasma and platelets. During ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Byrnes JR,Wolberg AS

    更新日期:2016-06-01 00:00:00

  • Management of musculoskeletal complications of hemophilia.

    abstract::Prophylactic treatment from ages 2 to 18 years could prevent the development of hemophilic arthropathy if the concentration of the patient's deficient factor is kept from falling below 1% of normal. Early treatment is of paramount importance because the immature skeleton is very sensitive to the complications of hemop...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Rodriguez-Merchan EC

    更新日期:2003-02-01 00:00:00

  • Outcome of Major Hemorrhage at a Major Cardiothoracic Center in Patients with Activated Major Hemorrhage Protocol versus Nonactivated Protocol.

    abstract::This study aimed to determine the impact of major hemorrhage (MH) protocol (MHP) activation on blood administration and patient outcome at a UK major cardiothoracic center. MH was defined in patients (> 16 years) as those who received > 5 units of red blood cells (RBCs) in < 4 hours, or > 10 units in 24 hours. Data we...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Chang KW,Owen S,Gaspar M,Laffan M,Arachchillage DRJ

    更新日期:2021-02-01 00:00:00

  • Coagulation-associated enhancement of fibrinolytic activity via a neutralization of PAI-1 activity.

    abstract::Total fibrinolytic activity in the vasculature is finely tuned by the balance between tissue plasminogen activator and plasminogen activator inhibitor type 1 (PAI-1). Although PAI-1 targets plasminogen activators, it also reacts with other serine proteases such as thrombin and factor Xa. The latter was shown to intera...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Urano T,Ihara H,Suzuki Y,Takada Y,Takada A

    更新日期:2000-01-01 00:00:00

  • Fibrin D-dimer in thrombogenic disorders.

    abstract::Measurement of D-dimer (fibrin degradation product) is important for determining not only the activation of fibrinolysis but also the severity of a hypercoagulable state. However, fibrin degradation products are in variable, and the reactivity to cross-linked fibrin degradation products produced during fibrin degradat...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Matsuo T,Kobayashi H,Kario K,Suzuki S

    更新日期:2000-01-01 00:00:00

  • The Fibrinolytic Status in Liver Diseases.

    abstract::The liver is the main site of synthesis and/or clearance of proteins involved in fibrinolysis. Therefore, chronic liver disease, including cirrhosis, leads to altered plasma levels of fibrinolytic proteins. Historical studies using in vitro clot lysis assays suggested that patients with chronic liver disease had accel...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Leebeek FW,Rijken DC

    更新日期:2015-07-01 00:00:00

  • Prevention of venous thromboembolism in medical patients with thrombocytopenia or with platelet dysfunction: a review of the literature.

    abstract::Current guidelines for venous thromboembolism (VTE) primary prophylaxis are based on randomized clinical trials that exclude subjects at a potentially high bleeding risk. Thus no specific recommendation/algorithm for pharmacological prophylaxis in patients with thrombocytopenia and/or platelet dysfunction is available...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Tufano A,Guida A,Di Minno MN,Prisco D,Cerbone AM,Di Minno G

    更新日期:2011-04-01 00:00:00

  • Perturbation of protease inhibitors and substrates in inflammatory arthritis.

    abstract::Defective systemic fibrinolysis and articular persistence of fibrinlike material are well recognized in RA. Perturbation of the major plasma protease inhibitors, A1AT, A2MG, and AT III, was explored in RA, psoriatic arthritis, and Reiter's syndrome. Experimental evidence is presented and assessed with respect to the p...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Rothschild BM,Thompson LD,Pifer DD,Chesney CM

    更新日期:1985-10-01 00:00:00

  • Using Artificial Intelligence to Manage Thrombosis Research, Diagnosis, and Clinical Management.

    abstract::Thrombosis development in either arterial or venous system remains a major cause of death and disability worldwide. This poorly controlled in vivo clotting could result in many severe complications including myocardial infarction, venous thromboembolism, stroke, and cerebral venous thrombosis, to name a few. These con...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Mishra A,Ashraf MZ

    更新日期:2020-06-01 00:00:00

  • Laboratory diagnosis of antithrombin and heparin cofactor II deficiency.

    abstract::Antithrombin activity should probably be determined in persons with early onset of recurrent thrombosis, especially if a family history is present. The initial screening test should be a heparin cofactor assay optimized to reduce the contribution of heparin cofactor II. If the heparin cofactor assay is low, an antigen...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Tollefsen DM

    更新日期:1990-04-01 00:00:00

  • Angiostatin.

    abstract::The quiescent vascular system in the adult body represents the imbalanced net outcome of overproduction of endogenous angiogenesis inhibitors and reduced levels of angiogenic factors. While some endogenous inhibitors are expressed under physiological conditions, they can also be generated in association with tumor gro...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Cao Y,Xue L

    更新日期:2004-02-01 00:00:00

  • The statins: multifunctional antithrombotic and antineoplastic drugs.

    abstract::Statins are approved by the Food and Drug Administration (FDA) for the treatment of hypercholesterolemia and have shown remarkable activity in preventing cardiovascular morbidity and mortality. The versatility of statins is increasingly being appreciated, however, and lowering cholesterol is only one attribute among m...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Splichal JE,Stamm JA,Ornstein DL

    更新日期:2003-06-01 00:00:00

  • Aspects of the laboratory identification of von Willebrand disease in women.

    abstract::The increased prevalence of the laboratory diagnosis of von Willebrand disease (vWD) in women presenting with menorrhagia has raised concerns regarding certain specifics in vWD testing in women, including when vWD testing should be done in relation to menses and whether testing should be done while the patient is not ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Kouides PA

    更新日期:2006-07-01 00:00:00

  • Past, present and future considerations on low molecular weight heparin differentiation: an epilogue.

    abstract::This epilogue represents a final summary of the issues discussed and highlighted at the International Summit on Differentiation of Low Molecular Weight Heparins (LMWHs). International scientists and physicians presented and discussed the physicochemical differences between LMWHs, and concluded that different productio...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Fareed J,Haas S,Sasahar A

    更新日期:1999-01-01 00:00:00

  • Clinical utility of thromboelastography: one size does not fit all.

    abstract::Coagulation management requires the balancing of different components that contribute to clot formation. These components include the interactions between platelets, procoagulant, anticoagulant, and fibrinolytic factors. The cause of bleeding or thrombotic events is often multifactorial; however, the tests clinicians ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Wegner J,Popovsky MA

    更新日期:2010-10-01 00:00:00

  • Consequences of homocysteine export and oxidation in the vascular system.

    abstract::The risk for arteriosclerosis and thrombosis of patients with severe hyperhomocysteinemia is reduced by homocysteine-lowering therapy. Whether this is the case in patients with mild hyperhomocysteinemia remains to be proved. Another challenge for researchers is to establish a satisfying pathological mechanism of the v...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Blom HJ

    更新日期:2000-01-01 00:00:00

  • Coacute heparin: a new simple monotest for monitoring heparin treatment.

    abstract::Anti-factor Xa methods have been generally accepted for the monitoring of heparin treatment mainly due to their sensitivity to LMWH and excellent performance on automated equipment. When such equipment is not available, as in small laboratories or on the night shift, there is a need for a simple manual method. In the ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Friberger P,Krig E,Eriksson-Skoog L,Svensson S,Isacson C

    更新日期:1993-01-01 00:00:00

  • Standardization of the INR: how good is your laboratory's INR and can it be improved?

    abstract::The prothrombin time (PT) assay is the most clinically ordered coagulation test and is most often used for monitoring of vitamin K antagonist (VKA) therapy (e.g., warfarin), where results are expressed as an international normalized ratio (INR). The INR is in essence the patient's PT "mathematically adjusted" to a sta...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Favaloro EJ,Adcock DM

    更新日期:2008-10-01 00:00:00

  • Rational diagnosis of pulmonary embolism (RADIA PE) in symptomatic outpatients with suspected PE: an improved strategy to exclude or diagnose venous thromboembolism by the sequential use of a clinical model, rapid ELISA D-dimer test, perfusion lung scan,

    abstract::A prospective management decision analysis for the exclusion and diagnosis of pulmonary embolism (PE) based on pre-test clinical probability (PCP) estimation for PE, a rapid ELISA D-dimer test, perfusion lungscan (P-scan), CUS, spiral CT, and pulmonary angiography is proposed. The modified PCP model for PE of Wells et...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Michiels JJ

    更新日期:1998-01-01 00:00:00

  • Involvement of fibrinolytic factors in mid trimester amniotic fluid with development of severe early-onset preeclampsia.

    abstract::Impaired placental development is a well-known pathogenesis in preeclampsia. The present study was undertaken to elucidate the involvement of fibrinolytic factors in amniotic fluid in midtrimester with development of severe early-onset preeclampsia. Amniotic fluid was obtained by amniocentesis at 15 to 18 weeks of ges...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Adachi T,Nakabayashi M,Sakura M,Ando K

    更新日期:1999-01-01 00:00:00

  • Factor XI Deficiency.

    abstract::Severe factor XI (FXI) deficiency is an injury-related bleeding disorder common in Ashkenazi Jews and rare worldwide. In the past two decades, more than 180 mutations in the FXI gene have been reported in patients with FXI deficiency, five of which show a founder effect (Cys38Arg, Gln88Stop, Cys128Stop, Glu117stop, an...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Duga S,Salomon O

    更新日期:2009-06-01 00:00:00

  • Thrombosis in myeloproliferative neoplasms.

    abstract::Thrombotic events are very frequent and represent the main cause of morbidity and mortality in patients with Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), mainly polycythemia vera and essential thrombocythemia. Pathogenesis of blood clotting activation in these diseases is multifactorial, and i...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Falanga A,Marchetti M

    更新日期:2014-04-01 00:00:00

  • Vitamin K-dependent proteins bind to very low-density lipoproteins.

    abstract::It has been demonstrated that the surface of large VLDL Sf 100-400 can bind both prothrombin and Factor X(Xa) and that on VLDL Factor Xa can convert prothrombin to thrombin, which degrades apo B and apo E. It has been reported also that the VLDL kinetically supports the conversion of prothrombin to thrombin. The bindi...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Bradley WA,Gianturco SH

    更新日期:1988-07-01 00:00:00

  • New insights into the role of erythrocytes in thrombus formation.

    abstract::The role of erythrocytes in thrombus formation has previously been regarded as passive by their influence on rheology. Erythrocytes are known, due to their abundance and size, to push platelets to the vascular wall (laminar shearing). This results in an increased platelet delivery at the vascular wall enabling platele...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Du VX,Huskens D,Maas C,Al Dieri R,de Groot PG,de Laat B

    更新日期:2014-02-01 00:00:00

  • Hemostatic Efficacy of Pathogen-Inactivated Blood Components.

    abstract::Pathogen inactivation (PI), or pathogen reduction technology, reduces the infectious risk of plasma and platelet transfusions, and also affects clotting factor activities and platelet viabilities. Plasma is treated with solvent-detergent to disrupt enveloped viruses, or with photoactive agents methylene blue plus ligh...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Ramsey G

    更新日期:2016-03-01 00:00:00