Typically, FV deficiency is first suspected in a patient with bleeding symptoms who has a prolongation of both the prothrombin time and the partial thromboplastin time. If a low FV activity is discovered, then congenital FV deficiency must be distinguished from consumptive coagulopathy, liver disease, combined FV and FVIII deficiencies, and an acquired FV inhibitor.
The clinical setting is often sufficient to differentiate FV deficiency from disseminated intravascular coagulation or liver disease, but testing for d-dimers, fibrinogen level, and liver dysfunction or damage may be useful. A FVIII level is necessary to distinguish isolated FV deficiency from the combined deficiency of FV and FVIII and may help in distinguishing congenital FV deficiency from that owing to liver failure, as FVIII levels are often elevated in liver dysfunction.
Rare Coagulation Disorders Resource Room, an extension of the RBDD Registry (eu.rbdd.org), was developed in collaboration with the Indiana Hemophilia & Thrombosis Center (www.innovativehematology.org).
The Resource Room serves as a platform to promote international collaboration to increase knowledge of these very rare disorders and to improve the care of affected individuals. The international RBDD mission, under the leadership of Dr. Peyvandi, will continue to be expanded over time.
The Resource Room thanks all contributing authors of the website articles that have made this valuable information available to the global community of both individuals affected with rare coagulation disorders and their healthcare providers.
