Abstract

Significance: Von Willebrand Disease (VWD) is a bleeding disorder characterized by congenital or acquired deficiency of von Willebrand Factor (VWF) leading to impaired clotting. The treatment involves VWF replacement with recombinant or plasma-derived formulations. Indications for replacement include active bleeding, surgery, or chronic prophylaxis.  A comprehensive formulary review was conducted in our health system in 2023 to streamline and standardize these agents. In addition, a standardized electronic order set was implemented to facilitate compliance with prescribing criteria including appropriate indication, dose, duration, and monitoring.

Purpose: The purpose of this project was to evaluate the utilization of these products in an acute care setting.

Methods: This was a retrospective, multi-site, observational chart review conducted across six hospitals within our health system from July 2023 through July 2025. Adult patients with a diagnosis of VWD who received at least one dose of recombinant VWF or plasma derived antihemophilic/von Willebrand factor complex (AHF/VWF) during hospitalization were included. Patients were excluded if they were younger than 18 years of age, pregnant, incarcerated, or had incomplete records. The data was extracted from the electronic health record into a secure, password-protected spreadsheet and de-identified prior to analysis. Collected variables included patient demographics, reason for admission, The primary outcomes were to assess compliance with the established prescribing criteria per the electronic order set and reason for factor product administration during hospital stay. The secondary outcomes were the incidence of hypersensitivity or infusion-related reactions, incidence of thromboembolic events (e.g., deep venous thrombosis, arterial thrombosis, pulmonary embolism, myocardial infarction, stroke, transient ischemic attack, etc.), and hospital length of stay (LOS).

Results: In total, twenty-eight patients were included. The most common indication for VWF replacement during acute care admission was surgical prophylaxis, accounting for 13/28 (46%) of cases. Plasma-derived AHF/WFA was administered in 68% of patients, while r-VWF was given to 32% of patients. Standardized electronic order set utilization was seen 68% of the time with appropriate dosing ordered in 57% of patients. Patients predominantly received fix dosing at 79% compared to weight-based dosing at 21%. Average total doses given during hospital LOS was 3 doses with average hospital LOS of 7 days. There were no reports of thromboembolic events or hypersensitivity noted.

Discussion: Further evaluation of patients who received sub-optimal dosing is needed to identify gaps in the pre-approved electronic order set. Furthermore, additional review of maintenance therapy would provide insight into the continued need for administration of VWF product during hospital stay.

Publication Date

Winter 12-10-2025

Presented At:

Midyear Clinical Meeting and Exhibition (ASHP)

Content Type

Poster

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