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Review
. 2021 Dec 22:12:20406207211064064.
doi: 10.1177/20406207211064064. eCollection 2021.

Prophylactic management of patients with von Willebrand disease

Massimo Franchini  1 , Omid Seidizadeh  2 , Pier Mannuccio Mannucci  2
Affiliations
Review

Prophylactic management of patients with von Willebrand disease

Massimo Franchini et al. Ther Adv Hematol. .

Abstract

Von Willebrand disease, the most common inherited bleeding disorder that affects both males and females, is due to quantitative or qualitative defects of the multimeric glycoprotein von Willebrand factor, which cause mucous membrane bleeding but also soft tissue bleeding owing to the secondary deficiency of factor VIII. The aim of treatment is to correct this dual defect of hemostasis. In addition to the episodic management of bleeding episodes, therapy includes their short- or long-term prevention. Short-term prophylaxis is mainly warranted in order to provide effective hemostatic coverage to patients undergoing surgery or invasive procedures and to affected women at the time of delivery or during menstruations associated with excessive bleeding. The aim of long-term prophylaxis is to prevent bleeding in particular categories of patients at increased risk of frequent and spontaneous bleeding in the joints, nose, and gastrointestinal tract.

Keywords: bleeding; long term; prophylaxis; short term; von Willebrand disease.

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Conflict of interest statement

Conflict of interest statement: The authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Massimo Franchini is consulting for Bayer Health Care, and Novo Nordisk. Pier Mannuccio Mannucci is member of the scientific board for the Bayer Awards. He has also received honoraria from Bayer, Kedrion, Roche, and Octapharma for lectures at educational symposia. Omid Seidizadeh has no conflicts to disclose.

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