We studied a Spanish family in which one of the female members presented recurrent
thrombophlebitis in both legs after three different deliveries. Biological and antigenic
activity of protein C was decreased (35% and 42% respectively). Reduced protein C
levels were also observed in 6 other family members. Administration of danazol (600
mg/day) in two patients with protein C deficiency elevated this protein and discontinuation
of the drug resulted in a reduction of protein C to pretreatment values. The proposita
showed a normal fibrinolytic activity and infusion of DDAVP produced a similar response
of FVIII/VWF and plasminogen activator to those observed in healthy subjects.
Keywords
Protein C deficiency - Recurrent thrombosis - Danazol - DDAVP