Promising coagulation factor VIII bypassing strategies for patients with haemophilia A

Blood Coagul Fibrinolysis. 2014 Sep;25(6):539-52. doi: 10.1097/MBC.0000000000000098.

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

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Blood Coagulation Factors / metabolism
  • Blood Coagulation Factors / therapeutic use
  • Blood Platelets / drug effects*
  • Blood Platelets / metabolism
  • Blood Platelets / pathology
  • Coagulants / metabolism
  • Coagulants / therapeutic use*
  • Factor VII / metabolism
  • Factor VII / therapeutic use
  • Factor VIII / metabolism
  • Factor VIII / therapeutic use
  • Factor VIIa / metabolism
  • Factor VIIa / therapeutic use
  • Factor X / metabolism
  • Factor X / therapeutic use
  • Factor Xa / metabolism
  • Factor Xa / therapeutic use
  • Hemophilia A / blood
  • Hemophilia A / drug therapy*
  • Hemophilia A / pathology
  • Hemostasis / drug effects
  • Humans
  • Platelet Activation / drug effects
  • Prothrombin / metabolism
  • Recombinant Proteins / metabolism
  • Recombinant Proteins / therapeutic use
  • Thrombin / metabolism

Substances

  • Blood Coagulation Factors
  • Coagulants
  • Recombinant Proteins
  • recombinant factor VIII SQ
  • Factor VII
  • Prothrombin
  • Factor VIII
  • Factor X
  • recombinant FVIIa
  • anti-inhibitor coagulant complex
  • Factor VIIa
  • Thrombin
  • Factor Xa