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Deficient activity of von Willebrand factor-cleaving protease in chronic
relapsing thrombotic thrombocytopenic purpura
M Furlan, R Robles, M Solenthaler, M Wassmer, P Sandoz and B Lammle
Central Hematology Laboratory, University Hospital, Inselspital, Bern,
Switzerland.
In patients with thrombotic thrombocytopenic purpura (TTP), excessive
intravascular platelet aggregation has been associated with appearance in
plasma of unusually large von Willebrand factor (vWF) multimers. These
extremely adhesive vWF multimers may arise due to deficiency of a
"depolymerase" cleaving vWF to smaller molecular forms, either by reducing
the interdimeric disulfide bridges or by proteolytic degradation. We
studied the activity of a recently described vWF- cleaving protease in four
patients with chronic relapsing TTP. Diluted plasma samples of TTP patients
were incubated with purified normal human vWF in the presence of a serine
protease inhibitor, at low ionic strength, and in the presence of urea and
barium ions. The extent of vWF degradation was assayed by electrophoresis
in sodium dodecyl sulfate-agarose gels and immunoblotting. Four patients,
that included two brothers, with chronic relapsing TTP displayed either
substantially reduced levels or a complete absence of vWF-cleaving protease
activity. In none of these patient plasmas was an inhibitor of or an
antibody against the vWF-cleaving protease established. Our data suggest
that the unusually large vWF multimers found in TTP patients may be caused
by deficient vWF-cleaving protease activity. Deficiency of this protease
may be inherited in an autosomal recessive manner and seems to predispose
to chronic relapsing TTP. The assay of the vWF-cleaving protease activity
may be used as a sensitive diagnostic tool for identification of subjects
with a latent TTP tendency.
Volume 89,
Issue 9,
pp. 3097-3103,
05/01/1997
Copyright © 1997 by The American Society of Hematology
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