Summary
A patient with two congenital defects, i.e. a severe factor V deficiency and an aortic coarctation is described. Arguments for a dominant mode of inheritance of the factor V deficiency are presented. A coinciding haemostatic defect consisting of a prolonged bleeding time, which could be corrected by fresh plasma and cryoprecipitate, and a decreased platelet retention in the glass bead test disappeared after surgical correction of the coarctation.
Extensive platelet studies failed to show any further defect.
