CLINICAL MANIFESTATIONS IN 28 ITALIAN AND IRANIAN PATIENTS WITH SEVERE FACTOR-VII DEFICIENCY

There has been wide variation in the reported haemorrhagic manifestati ons of factor VII deficiency. We examined type and frequency of clinic al manifestations in 28 Iranian and Italian patients with severe defic iency (factor VII coagulant activity 2% or less). The most frequent sy mptoms were epistaxis and menorrhagia, whereas soft tissue bleeding su ch as haemarthrosis and muscle haematoma was less frequent. Only 5 of 9 patient who underwent surgery without factor VII replacement therapy had postoperative bleeding severe enough to require blood transfusion . No thrombotic manifestation occurred. A factor VII functional assay based on the use of human thromboplastin was a better predictor of the bleeding tendency of these patients than a rabbit thromboplastin-base d functional assay or immunoassay. On the whole, this study shows that in severe factor VII deficiency bleeding in mucosal tracts is not unc ommon. Surgery can sometimes be performed without replacement therapy and without haemorrhagic complications.