Inhibitors in Hemophilia A: Advances in Elucidation of Inhibitory Mechanisms and in Inhibitor Management with Bypassing Agents
Autor: | Natalya M Ananyeva, Timothy K Lee, Midori Shima, Nisha Jain, Evgueni L. Saenko |
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Rok vydání: | 2009 |
Předmět: |
Antibodies
Catalytic Enzyme-Linked Immunosorbent Assay Hemorrhage Factor VIIa Hemophilia A Bioinformatics Epitopes Peptide Library hemic and lymphatic diseases Activated factor VII Coagulopathy Animals Humans Medicine Adverse effect Factor VIII Blood Coagulation Factor Inhibitors business.industry Treatment regimen Treatment options Hematology medicine.disease Inhibitory antibodies Prothrombin complex concentrate Blood Coagulation Factors Recombinant Proteins Protein Structure Tertiary Thrombelastography Immunology Cardiology and Cardiovascular Medicine business Epitope Mapping medicine.drug |
Zdroj: | Seminars in Thrombosis and Hemostasis. 35:735-751 |
ISSN: | 1098-9064 0094-6176 |
DOI: | 10.1055/s-0029-1245106 |
Popis: | Development of inhibitory antibodies (inhibitors) to factor VIII (FVIII) is the most serious adverse event in replacement therapy of hemophilia A patients. The etiology and management of this condition remain major challenges for both researchers and clinicians. In the present review, we discuss recent advances in understanding the molecular mechanisms by which inhibitors inactivate FVIII and experimental approaches used for the mapping of inhibitor epitopes. We also present a comparative analysis of treatment of hemophilia A patients with inhibitors with currently available bypassing agents-activated prothrombin complex concentrate (FEIBA VH; Baxter Healthcare Corp., Westlake Village, CA) and recombinant activated factor VII (NovoSeven; Novo Nordisk, Princeton, NJ)-and describe some ongoing research programs aimed at developing new treatment options for these patients. Availability of sensitive and standardized laboratory assays that would assist in monitoring the effectiveness of bypass therapies is essential for designing customized treatment regimens and improvement in the management of health conditions of hemophilia patients with inhibitors. |
Databáze: | OpenAIRE |
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