Ramiro J. Núñez-Vázquez, Sección de Trombosis y Hemostasia, Hospital Universitario Virgen del Rocío, Sevilla, España
Elsa López-Ansoar, Unidad de Trombosis y Hemostasia, Hospital Universitario Alvaro Cunqueiro, Vigo (Pontevedra), España
Manuel Rodríguez-López, Servicio de Hematología y Hemoterapia, Hospital Universitario Álvaro Cunqueiro, EOXI Vigo, Vigo, Pontevedra, España
Von Willebrand disease is the most common bleeding disorder, often underdiagnosed, affecting both men and women. Diagnostic advances, including molecular biology, have improved detection. Traditional treatment is on-demand, but prophylaxis is gaining relevance, especially in severe or recurrent cases, following the hemophilia model. The latest guidelines recommend prophylaxis in patients with a history of severe and frequent bleeding, but evidence remains limited, supported by various retrospective studies. The PRO.WILL study demonstrated a significant reduction in the risk and frequency of bleeding with prophylaxis compared to on-demand treatment. Other studies support the efficacy of Wilate® and Veyvondi®, FVW/FVIII concentrates, although results vary depending on the type of von Willebrand disease and tolerability. More randomized controlled clinical trials are needed to better evaluate prophylaxis versus on-demand treatment, especially in mucosal bleeding and other scenarios.
Keywords: Von Willebrand disease. Prophylaxis. Hemophilia model. Retrospective studies. Randomized controlled trials.