CONGENITAL FACTOR-VII DEFICIENCY - REPORT OF 4 NEW CASES

Four new cases with congenital homozygous factor VII deficiency were described. Factor VII levels were reduced to < 1%, 3%, 8% and 10%, respectively. The incidence and severity of bleeding symptoms were well correlated with the measured factor VII activity. In the severe case of factor VII deficiency (< 1%) a home treatment program was started because of severe recurrent hemarthroses. This entailed transfusions of 20 U[units]/kg body wt prothrombin complex or factor VII concentrate in case of acute bleeding approximately every 3 wk. These transfusions were carried out successfully without any problems. In contradiction, 2 brothers with hypoproconvertinemia (factor VII 8% and 10%, respectively) reached an age of more than 70 yr. Despite replacement therapy, postoperative bleeding followed 1 appendectomy, whereas no postoperative bleeding followed patients requiring Achilles tendon lengthening and an above knee amputation and only slight bleeding followed a tonsillectomy. Patients with factor VII deficiency of less than 10%, when undergoing surgery, should maintain a minimal factor VII activity of 10-15% during the first 3 postoperative days.