Monitoring of von Willebrand factor inhibitors in patients with type 3 von Willebrand disease using a quantitative assay.

ABSTRACT:

Background

Antibodies inhibiting von Willebrand factor (VWF) develop in a subset of patients with type 3 von Willebrand disease (VWD3) and may be detected by their inhibition of ristocetin cofactor activity (VWF:RCo). Some also inhibit factor VIII activity (VIII:C).

Aim

To describe monitoring of ten VWD3 patients for VWF inhibitors using a quantitative assay.

Methods

VWF inhibitor was measured by comparing VWF:RCo activity of a mix of patient and pooled normal plasma (PNP) with a mix of buffer and PNP, using agglutination of fixed normal platelets in microtiter plates or lyophilized platelets in an aggregometer. VIII:C inhibitor was measured by Bethesda assay. Preanalytical heat treatment of patient plasma was used during treatment episodes.

Results

Four of 10

SUBMITTER: Miller CH 

PROVIDER: S-EPMC8667261 | biostudies-literature | 2021 Sep

REPOSITORIES: biostudies-literature