H1N1 Influenza (Swine Flu)-Associated Thrombotic Microangiopathy with a Markedly High Plasma Ratio of von Willebrand Factor to ADAMTS13

DOI Web Site 4 Citations 16 References
  • Akiyama Rui
    Department of Pediatrics, Matsudo City Hospital Children's Medical Centre, Japan
  • Komori Isao
    Department of Pediatrics, Matsudo City Hospital Children's Medical Centre, Japan
  • Hiramoto Ryugo
    Department of Pediatrics, Matsudo City Hospital Children's Medical Centre, Japan
  • Isonishi Ayami
    Department of Blood Transfusion Medicine, Nara Medical University, Japan
  • Matsumoto Masanori
    Department of Blood Transfusion Medicine, Nara Medical University, Japan
  • Fujimura Yoshihiro
    Department of Blood Transfusion Medicine, Nara Medical University, Japan

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Abstract

We describe an 18-year-old woman infected with H1N1 influenza followed by thrombotic microangiopathy. During the acute phase, her plasma levels of von Willebrand factor (VWF) were remarkably elevated, whereas those of ADAMTS13 were reduced without its inhibitors, generating a markedly high ratio of VWF to ADAMTS13 in circulation. A retrospective analysis established the following hypothesis: an influenza-mediated cytokine storm induced an enhanced release of unusually large VWF multimers (UL-VWFM) from vascular endothelial cells, generating platelet thrombi in microcirculatures under high shear stress. Plasma exchange removed UL-VWFM and cytokines, and rescued her life. This report sheds a light on a hitherto unrecognized influenza complication.<br>

Journal

  • Internal Medicine

    Internal Medicine 50 (6), 643-647, 2011

    The Japanese Society of Internal Medicine

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