Analysis of current perioperative management with Haemate((R)) P/Humate P-(R) in von Willebrand disease: Identifying the need for personalized treatment

作者：Hazendonk H C A M; Heijdra J M; de Jager N C B; Veerman H C; Boender J; van Moort I; Mathot R A A; Meijer K; Laros van Gorkom B A P; Eikenboom J; Fijnvandraat K; Leebeek F W G; Cnossen M H^*

来源：Haemophilia, 2018, 24(3): 460-470.

DOI：10.1111/hae.13451

• individualized medicine
• surgery
• therapy
• von Willebrand Disease
• von Willebrand factor (MESH entry database)

IntroductionPatients with Von Willebrand disease (VWD) are regularly treated with VWF-containing concentrates in case of acute bleeding, trauma and dental or surgical procedures.
AimIn this multicentre retrospective study, current perioperative management with a von Willebrand factor (VWF)/Factor VIII (FVIII) concentrate (Haemate((R)) P) in patients with VWD was evaluated.
Patients/MethodsPatients with VWD undergoing minor or major surgery between 2000 and 2015, requiring treatment with a VWF/FVIII concentrate (Haemate((R)) P), were included. Achieved VWF activity (VWF:Act) and FVIII during FVIII-based treatment regimens were compared to predefined target levels in national guidelines.
ResultsIn total, 103 patients with VWD (148 surgeries) were included: 54 type 1 (73 surgeries), 43 type 2 (67 surgeries) and 6 type 3 (8 surgeries). Overall, treatment resulted in high VWF:Act and FVIII levels, defined as 0.20IU/mL above predefined levels. In patients with type 1 VWD, respectively, 65% and 91% of trough VWF:Act and FVIII levels were higher than target levels. In patients with type 2 and type 3 VWD, respectively, 53% and 57% of trough VWF:Act and 72% and 73% of trough FVIII levels were higher than target level. Furthermore, FVIII accumulation over time was observed, while VWF:Act showed a declining trend, leading to significantly higher levels of FVIII than VWF:Act.
ConclusionHigh VWF:Act and accumulation of FVIII were observed after perioperative FVIII-based replacement therapy in patients with VWD, both underlining the necessity of personalization of dosing regimens to optimize perioperative treatment.

• 出版日期2018-5