Deficient Activity of Von Willebrand Factor-cleaving Protease in Patients with Upshaw-Schulman Syndrome

Overview

Journal Int J Hematol

Specialty Hematology

Date 2001 Sep 4

PMID 11530799

Citations 5

Authors

Y Sasahara

S Kumaki

Y Ohashi

M Minegishi

H Kano

F Bessho

S Tsuchiya

Affiliations

Soon will be listed here.

Abstract

We identified unusually large von Willebrand factor (vWF) multimers caused by deficient activity of vWF-cleaving protease in 2 patients with Upshaw-Schulman syndrome. The autoantibodies that inhibited the protease activity were not detected in the plasma of either patient. Periodic fresh-frozen plasma transfusion was effective for management of the hemolysis and thrombocytopenia. We detected enriched enzyme activity in a particular plasma fraction, although molecular cloning of this specific protease is needed to determine a more detailed pathogenesis and to develop new therapeutic approaches.

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