ABSTRACT
Clinical management of von Willebrand disease (or von Willebrand disorder [vWD]) often
involves factor replacement or desmopressin acetate (DDAVP) therapy to control (potential)
bleeding. Laboratory monitoring involves testing patient samples prior to therapy
and at discreet time points after therapy. Classical testing generally comprises assays
for factor VIII:coagulant activity, von Willebrand factor (vWF):antigen and vWF:ristocetin
cofactor activity. The PFA-100 (platelet function analyser) is a relatively new tool
for the investigation of primary hemostasis, and studies have shown its potential
utility in identifying both vWD and platelet disorders, and in monitoring DDAVP therapy
in these patients. However, the PFA-100 has limited utility in monitoring factor replacement
therapy. The collagen-binding activity (vWF:CB) assay is a relatively new functional
vWF assay and studies have also shown its utility in identifying vWD, and in monitoring
both DDAVP and factor replacement therapy in these patients. This review assesses
the laboratory monitoring of therapy for vWD with a special focus on the combined
potential utility of the PFA-100 and a vWF:CB assay sensitive for the presence or
absence of large vWF multimers. This review should be of value to both hemostasis
scientists and clinical specialists.
KEYWORDS
PFA-100 - von Willebrand factor (vWF) - von Willebrand disease - von Willebrand disorder
(vWD) - laboratory monitoring - therapy - desmopressin (DDAVP) - review
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Dr. Emmanuel J Favaloro
Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR),
SWAHS, Westmead
New South Wales, 2145, Australia
Email: emmanuel@icpmr.wsahs.nsw.gov.au