Deficient activity of von willebrand factor-cleaving protease in patients with upshaw-schulman syndrome

Yoji Sasahara, Satoru Kumaki, Yoshiyuki Ohashi, Masayoshi Minegishi, Hirotsugu Kano, Fumio Bessho, Shigeru Tsuchiya

Research output: Contribution to journalArticlepeer-review

21 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)109-114
Number of pages6
JournalInternational journal of hematology
Volume74
Issue number1
DOIs
Publication statusPublished - 2001 Jan 1

Keywords

  • Chronic relapsing thrombotic thrombocytopenic purpura; von Willebrand factor multimer
  • Upshaw-Schulman syndrome
  • Von Willebrand factor-cleaving protease

ASJC Scopus subject areas

  • Hematology

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