The use of activated recombinant coagulation factor VII during haemarthroses and synovectomy in a patient with congenital severe factor V deficiency.

Factor V deficiency is a rare hereditary bleeding disorder. Currently, FV concentrates are not available, and the treatment of spontaneous bleeding or bleeding associated with invasive procedures is transfusion of fresh frozen plasma (FFP). However, FFP transfusion can lead to the development of inhibitor to FV, and is associated with several potential transfusion reactions including allergic reactions. We report a patient with congenital severe FV deficiency with repeated haemarthroses of a shoulder joint, and progressively severe allergic reactions to FFP transfusions. In addition, the patient also developed acute pulmonary oedema. Activated recombinant coagulation factor VII (rFVIIa) was used as an alternative haemostatic agent to FFP. We describe the use of rFVIIa in this patient during haemarthroses, synovectomy, and physiotherapy.