Low-dose intermittent factor replacement for post-operative haemostasis in haemophilia.

Recommendations for factor replacement therapy for postoperative haemostasis in haemophilia are often empirical and based on the physiological understanding of haemostatic requirements. This report describes the haemostatic management of patients with severe haemophilia undergoing major surgery using lower than usually recommended levels of factor replacement therapy. Eighteen adults (11 with FVIII and seven with FIX deficiency) with an average weight of 52 kg (range: 27-69) underwent 20 major surgical procedures. Factor concentrates were administered by intermittent bolus infusions. The dose of FVIII infused before surgery was 76 mu kg-1 (range: 51-113) and that of FIX was 77 mu 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 mu kg-1 day-1 (range: 20-46) of FVIII and 23 mu kg-1 day-1 (range: 12-42) of FIX was used resulting in mean trough levels of 36% (range: 12-62) and 34% (range: 11-52), respectively. After day 4, an average of 19 mu kg-1 day-1 (range: 15-25) of FVIII and 18 mu kg-1 day-1 (range: 10-37) of FIX was administered until wound healing. The average duration of factor replacement was 11 days (range: 8-16). The mean dose of factor concentrate per patient was 260 mu kg-1 (range: 179-338) of FVIII and 300 mu 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.

Low-dose activated factor IX complex concentrates (FEIBA(R) ) for post-operative haemostasis in a patient with high responding factor VIII inhibitors.

Replacement therapy in patients with severe haemophilia A is associated with the development of inhibitory antibodies in about 15% [1,2]. The presence of inhibitors of factor VIII greatly complicates and compromises the treatment of these patients because of the lack of any completely satisfactory product to treat them. Haemostatic management for surgery in patients with inhibitors is very difficult. A product frequently used to treat bleeding episodes in such patients is prothrombin complex concentrate (PCC) [3] or its activated derivative (APCC) [4]. Activated recombinant human factor VII is another option [5]. For both these modalities of treatment, there are no laboratory tests that can be used to monitor clinical efficacy [5, 6]. Porcine factor VII is therefore the preferred product for surgery in patients with high-responding factor VIII inhibitors [7]. Unfortunately, none of these products are readily available in most developing countries, including India. We report the management of a patient with high-responding factor VIII inhibitor using low doses of FEIBA(R) (Immuno, Austria) in the post-operative period.