The initial evaluation includes the prothrombin time (PT) and partial thromboplastin time (PTT), both of which will be prolonged.
If an inhibitor is suspected, its presence should be confirmed with a mixing study and the inhibitor titer should be determined with a Bethesda assay. Factor V activity is measured by a prothrombin clot assay. Normal ranges are laboratory specific and age dependent.
As with many other bleeding disorders, evaluation for acute events depends upon the clinical symptoms. In cases of suspected intracranial hemorrhage, head CT is often appropriate for initial evaluation.
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.
