Background: In 2006 Haberichter et al. pointed out the clinical utility of von Willebrand factor propeptide (vWFpp) in identifying patients with type 1 von Willebrand disease (vWD) with decreased von Willebrand factor (vWF) survival. In 2015 Sanders et al. reported, that vWFpp identified severe type 1 vWD with very low vWF levels in patients who had previously been classified as type 3 vWD underlining the diagnostic significance of vWFpp in classifying vWD patients. In 2018 O´Sullivan emphasized that enhanced vWF clearance may play an important role in the pathogenesis of vWD with direct therapeutic relevance.

Aims: Aim of the study was the evaluation of selected samples from patients with different types of vWD – Low Willebrand factor (LWF), type 1, 1C, 3, 2A, 2M as well as Acquired vWD (AQ-vWD) – with a new, rapid and specific vWFpp ELISA available since 2019.

Methods: Recently a CE-labeled vWFpp-assay (Senova GmbH, Erfurt, Germany) became available. The sandwich type ELISA employs precoated ready-to-use strips and requires a total of about 90 min for completion. It was applied on specimen from 34 patients.

Results: vWFpp was below the limit of detection in vWD type 3 cases (n=2). The vWD type 2M case disclosed a vWFpp/vWF:Ag-ratio close to 2.

[Figure 1: Distribution of vWFpp/vWF:Ag-ratios in remaining vWD types]

Conclusions: The vWFpp/vWF:Ag-ratio proves to be helpful in discriminating patients with a markedly increased vWF clearance (vWD type 1C, AQ-vWD and specific type 1 and type 2 cases). Identification of vWD patients with enhanced vWF clearance may contribute to optimize therapeutic regimens, especially considering the treatment-option with DDAVP.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/von-willebrand-factor-propeptide-useful-diagnostic-add-on-to-differentiate-von-willebrand-disease-based-upon-increased-von-willebrand-factor-clearance/