FVIII at the crossroad of coagulation, bone and immune biology: emerging evidence biological activities beyond hemostasis
Abstract
Hemophilia A is an X-linked hereditary disorder related to the deficiency of coagulation factor VIII (FVIII) leading to spontaneous bleeding episodes particularly into joints and muscles. FVIII deficiency has been associated with altered bone remodeling, dysregulated macrophage polarization, and inflammatory processes combined with neoformation of abnormal blood vessels. Treatment based on FVIII can lead to the development of inhibitors that render FVIII concentrate infusion ineffective. In this context, hemophilia has entered a new therapeutic era with the development of new drugs (e.g. Emicizumab) to restore the hemostatic balance by bypassing pathologic acquired antibodies. The present review aims to discuss the potential extrahemostatic functions of FVIII which may be crucial information for defining the future therapies in hemophilia.
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