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

Haemophilia. 2018 May;24(3):460-470. doi: 10.1111/hae.13451. Epub 2018 Mar 24.

Abstract

Introduction: Patients with Von Willebrand disease (VWD) are regularly treated with VWF-containing concentrates in case of acute bleeding, trauma and dental or surgical procedures.

Aim: In this multicentre retrospective study, current perioperative management with a von Willebrand factor (VWF)/Factor VIII (FVIII) concentrate (Haemate® P) in patients with VWD was evaluated.

Patients/methods: Patients with VWD undergoing minor or major surgery between 2000 and 2015, requiring treatment with a VWF/FVIII concentrate (Haemate® P), were included. Achieved VWF activity (VWF:Act) and FVIII during FVIII-based treatment regimens were compared to predefined target levels in national guidelines.

Results: In 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 ≥0.20 IU/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.

Conclusion: High 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.

Keywords: individualized medicine; surgery; therapy; von Willebrand Disease; von Willebrand factor (MESH entry database).

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Drug Combinations
  • Factor VIII / therapeutic use*
  • Female
  • Hemorrhage / complications
  • Humans
  • Male
  • Middle Aged
  • Perioperative Period*
  • Precision Medicine*
  • Retrospective Studies
  • von Willebrand Diseases / complications
  • von Willebrand Diseases / drug therapy*
  • von Willebrand Diseases / surgery*
  • von Willebrand Factor / therapeutic use*

Substances

  • Drug Combinations
  • factor VIII, von Willebrand factor drug combination
  • von Willebrand Factor
  • Factor VIII