H C A M Hazendonk1, J M Heijdra1, N C B de Jager2, H C Veerman1, J Boender3, I van Moort1, R A A Mathôt2, K Meijer4, B A P Laros-van Gorkom5, J Eikenboom6, K Fijnvandraat7, F W G Leebeek3, M H Cnossen1. 1. Department of Paediatric Haematology, Erasmus University Medical Centre - Sophia Children's Hospital Rotterdam, Rotterdam, Netherlands. 2. Hospital Pharmacy - Clinical Pharmacology, Academic Medical Centre Amsterdam, Amsterdam, Netherlands. 3. Department of Haematology, Erasmus University Medical Centre Rotterdam, Rotterdam, Netherlands. 4. Department of Haematology, University of Groningen, University Medical Centre Groningen, Groningen, Netherlands. 5. Department of Haematology, Radboud University Medical Centre, Nijmegen, Netherlands. 6. Department of Thrombosis and Haemostasis, Leiden University Medical Centre, Leiden, Netherlands. 7. Department of Paediatric Haematology, Academic Medical Centre Amsterdam, Amsterdam, Netherlands.
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.
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.
Authors: Nathan T Connell; Veronica H Flood; Romina Brignardello-Petersen; Rezan Abdul-Kadir; Alice Arapshian; Susie Couper; Jean M Grow; Peter Kouides; Michael Laffan; Michelle Lavin; Frank W G Leebeek; Sarah H O'Brien; Margareth C Ozelo; Alberto Tosetto; Angela C Weyand; Paula D James; Mohamad A Kalot; Nedaa Husainat; Reem A Mustafa Journal: Blood Adv Date: 2021-01-12
Authors: Laura H Bukkems; Jessica M Heijdra; Nico C B de Jager; Hendrika C A M Hazendonk; Karin Fijnvandraat; Karina Meijer; Jeroen C J Eikenboom; Britta A P Laros-van Gorkom; Frank W G Leebeek; Marjon H Cnossen; Ron A A Mathôt Journal: Blood Adv Date: 2021-03-09
Authors: Nico C B de Jager; Laura H Bukkems; Jessica M Heijdra; Carolien H C A M Hazendonk; Karin Fijnvandraat; Karina Meijer; Jeroen Eikenboom; Britta A P Laros-van Gorkom; Frank W G Leebeek; Marjon H Cnossen; Ron A A Mathôt Journal: J Thromb Haemost Date: 2019-10-21 Impact factor: 5.824
Authors: Romina Brignardello-Petersen; Abdallah El Alayli; Nedaa Husainat; Mohamad Kalot; Shaneela Shahid; Yazan Aljabirii; Alec Britt; Hani Alturkmani; Hussein El-Khechen; Shahrzad Motaghi; John Roller; Ahmad Dimassi; Omar Abughanimeh; Bader Madoukh; Alice Arapshian; Jean M Grow; Peter Kouides; Michael Laffan; Frank W G Leebeek; Sarah H O'Brien; Alberto Tosetto; Paula D James; Nathan T Connell; Veronica Flood; Reem A Mustafa Journal: Blood Adv Date: 2022-01-11