Hemophilia and Hemostasis

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منابع مشابه

THROMBOSIS AND HEMOSTASIS Engineered factor IX variants bypass FVIII and correct hemophilia A phenotype in mice

1Institute for Transfusion Medicine and Immune Hematology, German Red Cross Blood Donor Service Baden-Wuerttemberg–Hessen, Clinics of the Johann Wolfgang Goethe University, Hessen, Germany; 2Children’s Hospital of Philadelphia, Philadelphia, PA; 3Hemophilia Center Frankfurt, Clinics of the Johann Wolfgang Goethe University, Hessen, Germany; and 4Institute for Biomedical Research Georg-Speyer-Ha...

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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Prevention and treatment of factor VIII inhibitors in murine hemophilia A

Inhibitory antibody formation is a major complication of factor VIII replacement therapy in patients with hemophilia A. To better understand the pathogenesis of this immunologic reaction, we evaluated the role of T-cell costimulatory signals for antifactor VIII antibody formation in a murine model of hemophilia A. Repeated intravenous injections of factor VIII in these factor VIII–deficient mic...

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THROMBOSIS AND HEMOSTASIS Proteolytic antibodies activate factor IX in patients with acquired hemophilia

1Centre de Recherche des Cordeliers, Université Pierre et Marie Curie, Paris, France; 2Université Paris Descartes, Unité Mixte de Recherche S872, Paris, France; 3Inserm, U872, Paris, France; 4Inserm U770 and Université Paris-Sud, Le Kremlin-Bicêtre, France; 5Inserm, U698, Université Paris 7, Paris, France; 6Université de Technologie de Compiègne, Centre National de la Recherche Scientifique, Un...

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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Mechanism of factor VIIa–dependent coagulation in hemophilia blood

The ability of factor VIIa to initiate thrombin generation and clot formation in blood from healthy donors, blood from patients with hemophilia A, and in anti–factor IX antibody–induced (“acquired”) hemophilia B blood was investigated. In normal blood, both factor VIIa–tissue factor (TF) complex and factor VIIa alone initiated thrombin generation. The efficiency of factor VIIa was about 0.0001 ...

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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Cutaneous wound healing is impaired in hemophilia B

We used a mouse model to test the hypothesis that the time course and histology of wound healing is altered in hemophilia B. Punch biopsies (3 mm) were placed in the skin of normal mice and mice with hemophilia. The size of the wounds was measured daily until the epidermal defect closed. All wounds closed in mice with hemophilia by 12 days, compared with 10 days in normal animals. Skin from the...

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ژورنال

عنوان ژورنال: Journal of Clinical Pathology

سال: 1982

ISSN: 0021-9746

DOI: 10.1136/jcp.35.8.905-e