Comparative Analysis of Pure VWF and VWF/FVIII Complex in Orthopedic Surgery for Type 2B von Willebrand Disease.

Abstract

BACKGROUND This report describes a 55-year-old man with hypertension, hypercholesterolemia, type 2B von Willebrand disease (VWD2B), and thrombocytopenia requiring 2 surgical total hip replacements managed with pure von Willebrand factor (VWF) concentrate (Wilfactin®) and VWF/Factor VIII (FVIII) complex concentrate (Voncento®). VWD2B is a rare, autosomal-dominant, inherited disorder of primary hemostasis caused by VWF alterations that increase its affinity for the GP1b receptor on circulating platelets. In some families, thrombocytopenia of varying severity is observed. This can worsen the hemorrhagic phenotype and require adapted management. Specific laboratory tests and genetic studies are used to confirm the diagnosis of VWD2B, particularly before surgery in which VWF concentrates may be required. CASE REPORT This patient underwent 2 hip replacement interventions, 3.5 months apart, performed by the same surgical team. Hemorrhage prevention was achieved using Wilfactin® for the first hip replacement procedure and required 9 injections of Wilfactin® (352.9 IU/kg). For the second hip replacement, which took place 3.5 months after, we used Voncento® to prevent bleeding and required 8 injections of Voncento® (282.3 IU/kg). Both treatments were effective in terms of bleeding prevention. No thrombotic event was reported. The hospital stay duration was identical for both interventions. CONCLUSIONS Here, we report a real-life comparison of Wilfactin® and Voncento® in the same patient and in the same peri-operative management conditions. This study confirms the efficacy and safety of these VWF concentrates in preventing bleeding without thrombotic risks and highlights the benefits of close laboratory monitoring to personalize treatment and optimize management.