ticlopidine-Associated Thrombotic Thrombocytopenic Purpura With an IgG-Type Inhibitor to von Willebrand Factor-Cleaving Protease Activity

  • Yasuhiro Sugio
  • Takashi Okamura
  • Kazuya Shimoda
  • Masanori Matsumoto
  • Hideo Yagi
  • Hiromichi Ishizashi
  • Yoshiyuki Niho
  • Shoichi Inaba
  • Yoshihiro Fujimura
Case Report

DOI: 10.1007/BF02982073

Cite this article as:
Sugio, Y., Okamura, T., Shimoda, K. et al. Int J Hematol (2001) 74: 347. doi:10.1007/BF02982073

Abstract

A 41-year-old Japanese man complained of a left-sided visual disturbance. Imaging by magnetic resonance angiography revealed a narrowing of the left internal cervical artery. Thus, ticlopidine (Tc) administration was started at a daily dose of 300 mg. However, 2 weeks later, severe thrombocytopenia, fever, nausea, and psychiatric symptoms developed; Tc was therefore discontinued. Based on the diagnostic hallmark of 5 clinical signs, the patient’s disease was diagnosed as thrombotic thrombocytopenic purpura (TTP). Daily plasmapheresis was performed for the first 4 days, and the patient’s clinical signs gradually improved. Von Willebrand factor-cleaving protease (vWF-CPase) activity in his plasma was less than 3% of that of the control sample at diagnosis, but that value recovered steadily following plasmapheresis. In addition, immunoglobulin G purified from the patient plasma inhibited vWF-CPase activity in normal plasma with a specific activity of 0.8 Bethesda units/mg. No sign of TTP relapse has been noted following cessation of Tc. Thus, it was concluded that the patient developed TTP by producing an inhibitory autoantibody against vWF-CPase activity that was presumably triggered by Tc administration.

Key words

von Willebrand factor-cleaving protease Autoantibody Antiplatelet therapy Plasma exchange Thrombotic thrombocytopenic purpura 

Copyright information

© The Japanese Society of Hematology 2001

Authors and Affiliations

  • Yasuhiro Sugio
    • 1
  • Takashi Okamura
    • 1
  • Kazuya Shimoda
    • 1
  • Masanori Matsumoto
    • 2
  • Hideo Yagi
    • 1
  • Hiromichi Ishizashi
    • 1
  • Yoshiyuki Niho
    • 1
    • 3
  • Shoichi Inaba
    • 4
  • Yoshihiro Fujimura
    • 2
  1. 1.First Department of Internal Medicine, Faculty of MedicineKyushu UniversityFukuoka
  2. 2.Blood Transfusion Medicine and Sports MedicineNara Medical UniversityNara
  3. 3.Chihaya HospitalFukuoka
  4. 4.Blood Transfusion DivisionKyushu University HospitalFukuokaJapan
  5. 5.Second Department of Internal MedicineKurume University School of MedicineKurumeJapan

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