This disorder causes a number of alterations in hematologic functioning. One of these is that factor VIII production is decreased, which has a negative effect on the body’s ability to regulate secretion (Whelan et al., 2013). The second alteration is that immunoglobulin binding protein production is impaired, which makes it difficult for blood clots to remain stable (Whelan et al., 2013). These two alterations are linked, and this has an effect on the interaction of factor VIII and von Willebrand factor, which is associated with the assembly of heavy and light chains found in factor FVIII and promotes effective and efficient secretion of factor VIII (Whelan et al., 2013). Von Willebrand factor also helps to stabilize factor FVIII and protects it from degradation, promoting the standard half-life of factor FVIII from two hours to twelve hours (Gouw et al., 2013).
The main alteration that this disorder causes is changes to the clotting cascade or the coagulation system. The main purpose of this system is to produce a stable fibrin clot that prevents excess bleeding at the site of an injury (Gouw et al., 2013). Without a stable fibrin clot, the individual with this disorder cannot sufficiently stop bleeding as the clot will dissolve and not be effective, meaning that they are in danger of excessive bleeding and bruising (Gouw et al., 2013). This is due to the deficiency in FVII, which is a vital part of the coagulation system.
Another alteration in the hematologic system caused by this disorder is that sufferers might hemorrhage into their joints, causing pain and inflammation (Gouw et al., 2013). This is caused by the fact that the cells in synovial fluid are producers of tissue factor pathway inhibitor, which leads to increases in the amount of factor Xa in the joints (Gouw et al., 2013), another key part of the coagulation pathway. This means that the hemophilic joints are more likely to bleed.
- Gouw, S. C., van der Bom, J. G., Ljung, R., Escuriola, C., Cid, A. R., Claeyssens-Donadel, S., … others. (2013). Factor VIII products and inhibitor development in severe hemophilia A. New England Journal of Medicine, 368(3), 231–239.
- Whelan, S. F., Hofbauer, C. J., Horling, F. M., Allacher, P., Wolfsegger, M. J., Oldenburg, J., … others. (2013). Distinct characteristics of antibody responses against factor VIII in healthy individuals and in different cohorts of hemophilia A patients. Blood, 121(6), 1039–1048.
