Abstract
Haemophilia A is an X-linked recessive monogenic hereditary bleeding disorder caused by a deficiency or functional defect in coagulation factor VIII (FVIII). Typically, only 30% haemophilia A patients are treated with FVIII-specific products successfully. Therefore, other promising clotting factors and FVIII-bypassing factors exhibiting sufficient FVIII-independent activity, low immunogenicity and prolonged half-life are needed to conquer this malady. Here, we will systematically review the current status of the diverse FVIII-bypassing factors for the treatment of FVIII-insensitive haemophilia A patients.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Blood Coagulation Factors / metabolism
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Blood Coagulation Factors / therapeutic use
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Blood Platelets / drug effects*
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Blood Platelets / metabolism
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Blood Platelets / pathology
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Coagulants / metabolism
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Coagulants / therapeutic use*
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Factor VII / metabolism
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Factor VII / therapeutic use
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Factor VIII / metabolism
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Factor VIII / therapeutic use
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Factor VIIa / metabolism
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Factor VIIa / therapeutic use
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Factor X / metabolism
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Factor X / therapeutic use
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Factor Xa / metabolism
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Factor Xa / therapeutic use
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Hemophilia A / blood
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Hemophilia A / drug therapy*
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Hemophilia A / pathology
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Hemostasis / drug effects
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Humans
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Platelet Activation / drug effects
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Prothrombin / metabolism
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Recombinant Proteins / metabolism
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Recombinant Proteins / therapeutic use
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Thrombin / metabolism
Substances
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Blood Coagulation Factors
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Coagulants
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Recombinant Proteins
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recombinant factor VIII SQ
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Factor VII
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Prothrombin
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Factor VIII
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Factor X
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recombinant FVIIa
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anti-inhibitor coagulant complex
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Factor VIIa
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Thrombin
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Factor Xa