Successful surgical haemostasis in patients with von Willebrand disease with Koate®DVI

Summary. This report describes our experience with Koate^®DVI, a factor VIII (FVIII) concentrate containing von Willebrand factor (VWF) for surgery in patients with von Willebrand’s disease (VWD). Twenty‐one patients underwent 26 procedures, 10 of which were major and 16 were minor. The median age was 27 years (3–55) and the mean weight was 52 kg (16–88). Among the ten patients (type 2–5; type 3–5) who underwent major procedures, the pre‐operative dose was 35 IU kg^?1 of FVIII followed by 10–20 IU kg^?1 once daily depending on FVIII:C levels. The mean total dose of FVIII used per procedures was 106 IU kg^?1 (30–190) over a mean duration of 7 days (3–11). In this group, pre‐infusion FVIII:C, VWF:Ag and VWF: ristocetin cofactor (RCoF) level that were 19.5% (1–64), 20 U dL^?1 (0–96) and 12% (0–66) increased to 72% (54–198), 131 U dL^?1 (68–206) and 68% (27–108) postinfusion, respectively. Sixteen minor procedures were performed in 11 patients (type 1–3, type 2–6, type 3–2). The preparative dose of FVIII was 10–20 IU kg^?1. The average duration of factor support was 2 days (1–3) for a mean total dose of 23 IU kg^?1 (9–60). The pre‐infusion levels of FVIII:C, VWF:Ag and VWF:ristocetin cofactor (RCo) which were 31% (22–64), 25.5 U dL^?1 (0–63) and 21% (0–76), respectively, increased to 76% (27–111), 73 U dL^?1 (30–137) and 45% (2–106) postinfusion. Whereas surgical haemostasis was achieved in all patients, minor postoperative bleeding occurred after one procedure in each group. Both were controlled with additional doses of factor replacement. We conclude that Koate^®DVI in modest doses provide adequate haemostasis for surgery in patients with VWD.