Inhibitors in congenital coagulation disorders

The Danger Model: A Renewed Sense of Self

2002 • 4,107 citations

Natural versus adaptive regulatory T cells

2003 • 1,363 citations

Homeostasis and Self-Tolerance in the Immune System: Turning Lymphocytes off

1998 • 989 citations

Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis.

2001 • 772 citations

Definitions in Hemophilia

2001 • 708 citations

The Nijmegen Modification of the Bethesda Assay for Factor VIII:C Inhibitors: Improved Specificity and Reliability

1995 • 640 citations

Abnormal histidine metabolism in thyrotoxicosis in man. A possible index of impaired folate function.

1965 • 611 citations

The epidemiology of inhibitors in haemophilia A: a systematic review

2003 • 603 citations

The expanding B7 superfamily: Increasing complexity in costimulatory signals regulating T cell function

2001 • 413 citations

Cost of care and quality of life for patients with hemophilia complicated by inhibitors: the COCIS Study Group

2003 • 367 citations

A two-step, two-signal model for the primary activation of precursor helper T cells

1999 • 356 citations

Recombinant factor VIIa: review of efficacy, dosing regimens and safety in patients with congenital and acquired factor VIII or IX inhibitors

2004 • 312 citations

Multicenter Retrospective Study on the Utilization of FEIBA in France in Patients with Factor VIII and Factor IX Inhibitors

1997 • 287 citations

Rare Bleeding Disorder Registry: deficiencies of factors II, V, VII, X, XIII, fibrinogen and dysfibrinogenemias

2004 • 271 citations

The North American Immune Tolerance Registry: Practices, Outcomes, Outcome Predictors

2002 • 269 citations

The incidence of factor VIII and factor IX inhibitors in the hemophilia population of the UK and their effect on subsequent mortality, 1977–99

2004 • 266 citations

The Malmö International Brother Study (MIBS): further support for genetic predisposition to inhibitor development

2001 • 202 citations

Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia

2004 • 201 citations

HLA Genotype of Patients with Severe Haemophilia A due to Intron 22 Inversion with and without Inhibitors of Factor VIII

1997 • 195 citations

A Higher than Expected Incidence of Factor VIII Inhibitors in Multitransfused Haemophilia A Patients Treated with an Intermediate Purity Pasteurized Factor VIII Concentrate

1993 • 189 citations

Intensive exposure to factor VIII is a risk factor for inhibitor development in mild hemophilia A

2003 • 185 citations

The Inhibitor Antibody Response Is More Complex in Hemophilia A Patients Than in Most Nonhemophiliacs With Factor VIII Autoantibodies

1997 • 180 citations

A sudden increase in factor VIII inhibitor development in multitransfused hemophilia A patients in The Netherlands. Dutch Hemophilia Study Group

1993 • 179 citations

Incidence of factor VIII inhibitors in severe haemophilia: the importance of patient age

2001 • 178 citations

Prevention and treatment of factor VIII inhibitors in murine hemophilia A

2000 • 172 citations

A sudden increase in factor VIII inhibitor development in multitransfused hemophilia A patients in The Netherlands. Dutch Hemophilia Study Group

1993 • 172 citations

The natural history of factor VIII:C inhibitors in patients with hemophilia A: a national cooperative study. II. Observations on the initial development of factor VIII:C inhibitors

1988 • 171 citations

Factor VIII Inhibitors in Previously Treated Haemophilia A Patients with a Double Virus-inactivated Plasma Derived Factor VIII Concentrate

1997 • 168 citations

Environmental and genetic factors influencing inhibitor development

2004 • 163 citations

Anti-factor VIII antibodies of hemophiliac patients are frequently directed towards nonfunctional determinants and do not exhibit isotypic restriction

1993 • 155 citations

Natural antibodies to factor VIII (anti-hemophilic factor) in healthy individuals.

1992 • 141 citations

A Detailed Comparison of the Performance of the Standard versus the Nijmegen Modification of the Bethesda Assay in Detecting Factor VIII:C Inhibitors in the Haemophilia A Population of Canada

1998 • 140 citations

Residues 484-508 Contain a Major Determinant of the Inhibitory Epitope in the A2 Domain of Human Factor VIII

1995 • 137 citations

A new approach to treatment of bleeding episodes in young hemophilia patients: a single bolus megadose of recombinant activated factor VII (NovoSeven®)

2003 • 136 citations

Pathogenic antibodies to coagulation factors. Part one: Factor VIII and Factor IX

2004 • 133 citations

Factor VIII inhibitors in mild and moderate‐severity haemophilia A

1998 • 132 citations

The Molecular Basis of Hemophilia A: Genotype-Phenotype Relationships and Inhibitor Development

2003 • 132 citations

Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency

2003 • 131 citations

Localization of epitopes for human factor VIII inhibitor antibodies by immunoblotting and antibody neutralization

1989 • 130 citations

Age at first treatment and immune tolerance to factor VIII in severe hemophilia

2003 • 129 citations

The German Registry of immune tolerance treatment in hemophilia--1999 update.

2000 • 123 citations

Distribution of Th1- and Th2-induced Anti-factor VIII IgG Subclasses in Congenital and Acquired Hemophilia Patients

2002 • 121 citations

The Prevalence of Proteolytic Antibodies against Factor VIII in Hemophilia A

2002 • 112 citations

CD4+ T-cell clones specific for wild-type factor VIII: a molecular mechanism responsible for a higher incidence of inhibitor formation in mild/moderate hemophilia A

2003 • 111 citations

Treatment of coagulation inhibitors with extracorporeal immunoadsorption (Ig‐Therasorb)

2001 • 109 citations

Recombinant Factor IX Recovery and Inhibitor Safety: A Canadian Post-licensure Surveillance Study

2002 • 106 citations

Neonatal or hepatocyte growth factor–potentiated adult gene therapy with a retroviral vector results in therapeutic levels of canine factor IX for hemophilia B

2003 • 105 citations

Improved hemostasis with superactive analogs of factor VIIa in a mouse model of hemophilia A

2003 • 100 citations

Antifactor VIII Antibody Inhibiting Allogeneic but not Autologous Factor VIII in Patients With Mild Hemophilia A

1999 • 90 citations

Sensitization of CD4+ T Cells to Coagulation Factor VIII: Response in Congenital and Acquired Hemophilia Patients and in Healthy Subjects

2000 • 90 citations

Preventing restimulation of memory B cells in hemophilia A: a potential new strategy for the treatment of antibody-dependent immune disorders

2004 • 86 citations

Neutralizing antiidiotypic antibodies to factor VIII inhibitors after desensitization in patients with hemophilia A.

1996 • 85 citations

The Role of Genetics in Inhibitor Formation

1999 • 83 citations

Inhibitors in hemophilia A

2004 • 82 citations

Use of Recombinant Factor VIla (NovoSeven®) in the Treatment of Two Patients with Type III von Willebrand’s Disease and an inhibitor against von Willebrand Factor

1996 • 82 citations

Immune Tolerance Induction in Hemophilia A: A Review

2003 • 78 citations

Surveillance for Factor VIII Inhibitor Development in the Canadian Hemophilia A Population Following the Widespread Introduction of Recombinant Factor VIII Replacement Therapy

1998 • 77 citations

Rituximab in the treatment of alloimmune factor VIII and IX antibodies in two children with severe haemophilia

2004 • 76 citations

Loss of Tolerance to Exogenous and Endogenous Factor VIII in a Mild Hemophilia A Patient With an Arg593 to Cys Mutation

1997 • 75 citations

High incidence of anti‐FVIII antibodies against non‐coagulant epitopes in haemophilia A patients: a possible role for the half‐life of transfused FVIII

1996 • 72 citations

Immune tolerance treatment in haemophilia patients with inhibitors: the Spanish Registry

2001 • 71 citations

Activated prothrombin complex concentrate (FEIBA^®) treatment during surgery in patients with inhibitors to FVIII/IX

2004 • 69 citations

Role of CD154 in the secondary immune response: the reduction of pre-existing splenic germinal centers and anti-factor VIII inhibitor titer

2000 • 68 citations

Immunosuppressive Treatment in Haemophiliacs with Inhibitors to Factor VIII and Factor IX

1976 • 67 citations

Novel therapeutic approach for hemophilia using gene delivery of an engineered secreted activated Factor VII

2004 • 65 citations

Comparative analysis of Y chromosome structure in Bos taurus and <i>B. indicu</i><i>s</i> by FISH using region-specific, microdissected, and locus-specific DNA probes

1997 • 62 citations

Successful use of recombinant factor VIIa in a patient with inhibitor secondary to severe factor XI deficiency

2002 • 62 citations

Utilization of Previously Treated Patients (PTPs), Noninfected Patients (NIPs), and Previously Untreated Patients (PUPs) in the Evaluation of New Factor VIII and Factor IX Concentrates

1999 • 61 citations

First and Second Generation Recombinant Factor VIII Concentrates in Previously Untreated Patients: Recovery, Safety, Efficacy, and Inhibitor Development

2002 • 59 citations

International Immune Tolerance Registry, 1997 Update

1999 • 59 citations

The Canadian Hemophilia Registry as the basis for a national system for monitoring the use of factor concentrates

1995 • 54 citations

Peptide decoys selected by phage display block in vitro and in vivo activity of a human anti-FVIII inhibitor

2003 • 52 citations

Immunoadsorption may provide a cost‐effective approach to management of patients with inhibitors to FVIII

2003 • 46 citations

Orthopaedic surgery in haemophilic patients with inhibitors: an overview

2002 • 41 citations

Degradation Products of Factor VIII Which Can Lead to Increased Immunogenicity

1999 • 40 citations

Low detection rate of antibodies to non-functional epitopes on factor VIII in patients with hemophilia A and negative for inhibitors by Bethesda assay

2003 • 38 citations

In vivo neutralization of a C2 domain–specific human anti–Factor VIII inhibitor by an anti-idiotypic antibody

2003 • 37 citations

Dominant C2 Domain Epitope Specificity of Inhibitor Antibodies Elicited by a Heat Pasteurized Product, Factor VIII CPS-P, in Previously Treated Hemophilia A Patients without Inhibitors

1998 • 33 citations

Is the incidence and prevalence of inhibitors greater with recombinant products? Yes

2004 • 33 citations

Factor VIII inhibitors: A clinical overview

1982 • 32 citations

The Type of Factor VIII Deficient Plasma Used Influences the Performance of the Nijmegen Modification of the Bethesda Assay for Factor VIII Inhibitors

2001 • 32 citations

Immune tolerance therapy dose as an outcome predictor

2003 • 28 citations

Views on methods for monitoring recombinant factor VIIa in inhibitor patients

2004 • 28 citations

Clinical Efficacy of Prothrombin Complex Concentrates and Recombinant Factor VIIa in the Treatment of Bleeding Episodes in Patients with Factor VIII and IX Inhibitors

1999 • 26 citations

Elevated Function of Blood Clotting Factor VIIa Mutants That Have Enhanced Affinity for Membranes

2001 • 25 citations

Virological safety of the purified factor IX concentrate, Replenine

1995 • 23 citations

Mapping and Functional Studies of Two Alloantibodies Developed in Patients with Type 3 von Willebrand Disease

2000 • 20 citations

The maintenance of tolerance after successful immune tolerance induction in hemophilia A and B: the North American Registry. Factor VIII/IX Subcommittee of the International Society for Thrombosis and Hemostasis.

2000 • 19 citations

CD4+ T Cells Specific for Factor VIII as a Target for Specific Suppression of Inhibitor Production

2001 • 19 citations

The Maintenance of Tolerance After Successful Immune Tolerance Induction in Hemophilia A and B: The North American Registry

2000 • 18 citations

Update on the mechanism of action and future of activated prothrombin complex concentrates.

2004 • 12 citations

Factor VII gene intronic mutation in a lethal factor VII deficiency: effects on splice-site selection

2003 • 11 citations

Alloantibody from a patient with severe von Willebrand disease inhibits von Willebrand factor-FVIII interaction

1997 • 10 citations

Peptide decoys selected by phage display block in vitro and in vivo activity of a human anti-FVIII inhibitor

2003 • 9 citations

Should gene therapy be used for newborns with hemophilia?

2003 • 4 citations

Higher immune tolerance (IT) success rate with monoclonal factor VIII (mFVIII) when compared to recombinant (rFVIII): An analysis of the North American Immune Tolerance Registry (NAITR)

1999 • 4 citations