ABSTRACT
The PFA-100 (platelet function analyser; Dade-Behring, Marburg, Germany) is a relatively
new tool for the investigation of primary hemostasis. Recent studies have shown its
utility as a screening tool for investigating various platelet disorders and possible
von Willebrand disorder (vWD), both in the initial investigation and in subsequent
therapeutic monitoring of desmopressin therapy. This article reviews current findings
with respect to the identification of vWD, and highlights both the benefits and the
limitations of its clinical utility. In brief, sensitivity to vWD types 2A, 2B, 2M,
and 3 is > 98%, but overall sensitivity to vWD (types 1, 2A, 2B, 2M, and 3 combined)
is ~85 to 90%. Ultimately, the high sensitivity of the PFA-100 to vWD and its simplicity
of use provide its greatest strengths. However, because it is a global test system,
and also sensitive to low hematocrit, low platelet counts, and platelet dysfunction
(both congenital and acquired; e.g., secondary to medication such as aspirin) it must
be recognized that the PFA-100 is neither specific for, nor predictive of, any particular
disorder (inclusive of vWD). Nevertheless, used appropriately, the PFA-100 can be
considered a worthwhile addition to the hemostasis laboratory involved in the diagnosis
or therapeutic monitoring of vWD, and a normal PFA-100 result can be used with some
confidence to exclude severe vWD.
KEYWORDS
Platelet function - PFA-100 - bleeding disorders - von Willebrand Factor (vWF) - von
Willebrand disease - von Willebrand disorder (vWD) - laboratory assessment - diagnosis
- review
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Dr.
Emmanuel J Favaloro
Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR)
Westmead Hospital, SWAHS, Westmead, New South Wales, 2145, Australia
eMail: emmanuel@icpmr.swahs.nsw.gov.au
