Low-dose intermittent factor replacement for post-operative haemostasis inhaemophilia

Recommendations for factor replacement therapy for postoperative haemostasi s in haemophilia are often empirical and based on the physiological underst anding of haemostatic requirements. This report describes the haemostatic m anagement of patients with severe haemophilia undergoing major surgery usin g lower than usually recommended levels of factor replacement therapy. Eigh teen adults (11 with FVIlI and seven with FIX deficiency) with an average w eight of 52 kg (range: 27-69) underwent 20 major surgical procedures. Facto r concentrates were administered by intermittent bolus infusions. The dose of FVIII infused before surgery was 76 u kg(-1) (range: 51-113) and that of FIX was 77 u kg(-1) (range: 50-104). The preoperative levels achieved were 107% (range: 80-180) and 73% (range: 60-90), respectively. Between days 1 and 3 after surgery, an average of 29 u kg(-1) day(-1) (range: 20-46) of FV III and 23 u kg(-1) day(-1) (range: 12-42) of FIX was used resulting in mea n trough levels of 36% (range: 12-62) and 34% (range: 11-52), respectively. After day 4, an average of 19 u kg(-1) day(-1) (range: 15-25) of FVIII and 18 u kg(-1) day(-1) (range: 10-37) of FIX was administered until wound hea ling. The average duration of factor replacement was 11 days (range: 8-16). The mean dose of factor concentrate per patient was 260 u kg(-1) (range: 1 79-338) of FVIII and 300 u kg(-1) (range: 183-524) of FIX. The total amount of factor used per patient ranged from 12 380 to 19 980 units of FVIII and 8000 to 23 600 units of FIX. Only one patient had post-operative bleeding which was due to a surgical cause. It is concluded that it may be possible to use much lower than recommended levels of factor replacement therapy for postoperative haemostasis in severe haemophilia.