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DR. ROCK:
Normally, an enzyme, a
metalloprotease, acts on those forms to form the
normal kind of von Willebrand factor that goes
around in the circulation. But in the presence
of some patients, including the congenital ones,
and in the series of Tsai and Furlan, they have
found an inhibitor present in the plasma that
blocks this conversion leaving them with these
more active forms of von Willebrand factor
circulating in the plasma.
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