Congenital Combined Deficiency of Factor V and Factor VIII

Abstract

SummaryA case of congenital combined deficiency of factor V and factor VIII was reported. The patient, a 9 year old boy, gave a history of epistaxis, hematomas, and of hemorrhages following dental extraction since the age of 2 ; plasma levels of factor V and factor VIII were found to be 16% and 8% of normal, respectively. No one in his family had a deficiency of either factor. The effects of transfusion of normal fresh plasma and whole blood from a patient with hemophilia A were studied. While the former were similar to the pattern as seen in classical hemophilia, the latter consisted of an immediate increase of factor V activity and a delayed increase of factor VIII activity, despite the fact that factor VIII activity was almost absent from the donor’s blood.