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von
Willebrand Disease
von Willebrand
Factor (vWF) is an important blood clotting protein involved in
both assisting platelet adhesion and stabilization of clotting factor
VIII.
In von
Willebrand Disease (vWD), there is either a partial quantitative
deficiency or shortage of vWF (classified as vWD Type 1) or a qualitative
or functional deficiencey (classified as vWD Type 2). vWD Type 3
is rare and charachterized by virtually complete deficiency of vWF.
The incidence of vWD worldwide is estimated at 1% to 3% but may
be more common since mild cases may remain undetected.
Historically,
a wide variety of assays have been used to characterize vWF. Currently
the most common methods are
- vWF:AG assay by
Laurell rocket electrophoreses, ELISA or Latex Immunoassay and
- Ristocetin Cofactor
agglutination (RiCoF) or Ristocetin induced platelet aggregation
(RIPA).
These
two methods in combination can identify vWD types 1 and 2A which
represent some 85% of vWD cases.
Recently,
the Collagen Binding Assay (CBA) has been mentioned as a useful
alternative to the Ristocetin Cofactor assay for detecting variants
of vWF with defective adhesive function. This is an ELISA-like method
based on the ability of vWF to bind collagen, which is associated
with higher molecular weight multimers of vWF. The CBA assay correlates
closely with vWF function and bleeding problems.
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