Perioperative Replacement Therapy for a Patient with Hereditary Factor V Deficiency

Abstract

A seven-year-old boy, who had a history of subcutaneous bleeding was scheduled to receive repairing of cleft palate. He showed no abnormalities in clinical laboratory data except prolongation in PT and APTT. The examination in blood coagulation factors revealed the extremely low factor V activity (below 1% of normal).Preoperatively the relationship between factor V activity and APTT was investigated. It was found that no blood coagulative abnormalities developed as far as APTT remained below 45 seconds.After induction of anesthesia, 20ml/kg(300ml) of FFP was administered with the equivalent amount of exsanguination to avoid the overexpansion of blood volume. Thereafter APTT was improved from 86 seconds to 40 seconds. Two hours surgery performed without any difficulties in hemostasis, and patient recovered uneventfully. Postoperatively APTT and factor V activity were repeatedly measured and FFP was given whenever 25% or less in factor V activity or longer than 50 seconds in APTT was occured. Perioperatively the replacement therapy with FFP is essential for the patients with factor V deficiency.It is very important to avoid the overexpansion of blood volume for the patient's safety, and to repeatedly measure the APTT for evaluation of effectiveness of blood coagulation.