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Enhanced proteolysis of plasma von Willebrand factor in thrombotic
thrombocytopenic purpura and the hemolytic uremic syndrome
PM Mannucci, R Lombardi, A Lattuada, P Ruggenenti, GL Vigano, T Barbui and G Remuzzi
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, University of
Milano, Italy.
To examine whether enhanced in vivo proteolysis of von Willebrand factor
(vWF) would account for the reported loss of larger multimers in acute
thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS), we
studied eight patients with acute TTP/HUS whose blood samples were
collected into an anticoagulant containing a cocktail of protease
inhibitors to impede in vitro proteolysis. In all, enhanced proteolytic
degradation of vWF was expressed as a relative decrease in the intact
225-Kd subunit of vWF and a relative increase in the 176-Kd fragment.
However, instead of the loss of larger forms of normal multimers reported
by other investigators, the plasma of all but one of our patients (whether
they had TTP or HUS) contained a set of larger than normal (supranormal)
multimers. Hence, although proteolytic fragmentation of vWF was enhanced
during acute TTP/HUS, this phenomenon was not associated with the loss of
larger multimers. In the five patients who survived the acute disease and
underwent plasma exchange (three with HUS and two with chronic relapsing
TTP), subunits and fragments returned to normal values, and supranormal
multimers were no longer detected in plasma. In conclusion, even though vWF
proteolysis is enhanced in acute TTP/HUS, it does not lead to loss of
larger multimers.
Volume 74,
Issue 3,
pp. 978-983,
08/15/1989
Copyright © 1989 by The American Society of Hematology
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