Wolfgang Miesbach: Should Prophylaxis in Type 3 VWD Follow the Bleeding Phenotype?
Wolfgang Miesbach, Head of Hemostasis/Hemophilia Center at Medical Clinic 2 at University Hospital Frankfurt, shared a post on LinkedIn about a recent article by Cédric Hermans, published in Therapeutic Advances in Hematology, adding:
“Should prophylaxis in type 3 VWD follow the bleeding phenotype?
Cedric Hermans, Emna Gouider Belhadjali and colleagues report long-term prophylaxis with Wilfactin, a pdVWF concentrate almost devoid of FVIII, in 7 patients with type 3 VWD.
The idea: Patients were grouped by the bleeding prophylaxis had to prevent. Joint bleeds depend mainly on FVIII, which the infused VWF stabilises from the patients own production.
Mucosal bleeds depend on VWF itself. Each groups dose was therefore guided by the relevant trough level, measured 48 h after a 60 IU/kg test dose.
Dosing algorithm:
Joint bleeds: 40 IU/kg – twice weekly if FVIII:C more or equal to 20 IU/dL, three times weekly if lower
Mucosal bleeds: 60 IU/kg – twice weekly if VWF:RCo more or equal to 10 IU/dL, three times weekly if lower
What patients actually received
Joint group: 44 IU/kg, 1.8 infusions a week (84 IU/kg/week)
Mucosal group: 59 IU/kg, 2.4 infusions a week (142 IU/kg/week)
Outcomes Median ABR fell from 52 to 4.3, an 83% reduction.
Joint group: ABR 1.1. FVIII:C stayed more or equal to 20 IU/dL at 48 h, so no FVIII had to be added.
Mucosal group: ABR 4.2. Two of four patients could not keep up three infusions a week.
No inhibitors and no thrombosis.
The cohort is small, the comparison is historical, and the study ran from 2005 to 2012.
Even so, tailoring both dose and frequency to the bleeding phenotype makes clinical sense.”
Title: Clinical efficacy and safety of von Willebrand factor concentrate almost devoid of factor VIII in long-term prophylaxis study in patients older than 6 years with severe von Willebrand disease
Authors: Cédric Hermans, Balkis Meddeb, Quentin Van Thillo, Alain Gadisseur, Françoise Bridey, Céline Henriet, Anna Klukowska, Emna Gouider
Other posts featuring Wolfgang Miesbach on Hemostasis Today.
-
Oct 28, 2026, 03:25Owen McCarty: Exploring Platelet Polyphosphates in Coagulation Under Flow
-
Oct 28, 2026, 03:19Arwa Z. Al-Riyami: Key Takeaways from the Blood Conference in Auckland
-
Oct 28, 2026, 03:14Kristopher Jonas: The Ethical Case for Compensated Plasma Donation
-
Oct 28, 2026, 03:09Amber Paratore Sanchez: Therapeutic Plasma Exchange in PTE Patients with Cold Agglutinins
-
Oct 28, 2026, 03:04Fonkou Steve: Overcoming Fear to Build a Lifelong Blood Donor
-
Oct 28, 2026, 03:00Joanne Marlor: NHS Blood and Transplant Opens Country’s Largest Donor Centre in Reading
-
Oct 27, 2026, 23:04Stefano Navarro: Hypertension Reprogrammes Megakaryocytes and Increases Platelet Responsiveness to Thrombin
-
Oct 27, 2026, 23:03Enrique M. San Norberto García: What Is the Best Treatment for Femoropopliteal in-Stent Restenosis
-
Oct 27, 2026, 23:01Mandy-Lyn Crispim: One Donation Can Help Save More Than One Life