Blood Transfusion Supplement 4 - Plasma-derived medicinal products: demand and clinical use (off subscription) (September)
Clinical use of factor VIII and factor IX concentrates
Authors:  Massimo Morfini, Antonio Coppola, Massimo Franchini, Giovanni Di Minno
Pages:  55-63
To cite this article:  Blood Transfus 2013; 11 Suppl 4: s55-63
Doi:  10.2450/2013.010s
Published online:  08/10/2013

Factor VIII (FVIII) and factor IX (FIX) are the cofactor and the pro-enzyme, respectively, acting in the tenase complex, a key mechanism of physiological haemostasis in which a phospholipide-dependent reaction produces the activation of factor X1. Mutations in the genes encoding for coagulation FVIII or IX, both located in the X chromosome, are responsible for haemophilia A and haemophilia B, respectively. These congenital bleeding disorders occur overall in approximately 1 in 5,000 male live births without racial predilection. However, the prevalence of haemophilia A is estimated 4-6 times higher than haemophilia B2-3. (...)
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