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Elevated Von Willebrand factor propeptide for the diagnosis of thrombotic microangiopathy and for predicting a poor outcome

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Abstract

Thrombotic microangiopathy (TMA) is associated with vascular endothelial cell injury and is sometimes linked with poor outcome. Von Willebrand factor (VWF) propeptide (VWFpp) is considered to be a marker of vascular endothelial cell injury. The plasma levels of VWF, VWFpp, and thrombomodulin (TM) were evaluated for their use in the diagnosis of TMA in 75 patients with TMA. There were 30 TMA patients with marked decreases in ADAMTS13 (TMA/ADAMTS13) and 45 without the decrease (TMA/other). The plasma levels of TM, VWF, and VWFpp values were significantly high in patients with TMA, especially TMA/other group. The plasma levels of TM and VWFpp were significantly high in non-survivor with TMA. In the TMA/other group, the plasma levels of VWFpp were negatively correlated with ADAMTS13 activity. The plasma levels of TM correlated with the renal function, but the plasma levels of VWFpp did not. A ROC analysis indicated that VWFpp and TM were useful markers for the prediction of a poor outcome. These findings suggest that VWFpp is an useful marker for the diagnosis of TMA and for the prediction of poor outcome.

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Acknowledgments

This work was supported in part by a Grant-in-Aid for Blood Coagulation Abnormalities from the Ministry of Health, Labor and Welfare of Japan. We thank Dr. Yoshihiro Fujimura and Dr Masanori Matsumoto of the Department of Blood Transfusion Medicine, Nara Medical University, Nara for the measurement of the inhibitor for ADAMTS13.

Conflict of interest

All authors disclose no financial or personal relationship with other people or organizations that could inappropriately influence their work.

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Authors and Affiliations

  1. Department of Hematology and Oncology, Mie University Graduate School of Medicine, Mie, Japan

    Naomi Ito-Habe & Naoyuki Katayama

  2. Department of Molecular and Laboratory Medicine, Mie University Graduate School of Medicine, Tsu, Mie, 514-8507, Japan

    Hideo Wada & Tsutomu Nobori

  3. Transfusion Service, Mie University Hospital, Mie, Japan

    Takeshi Matsumoto & Kohshi Ohishi

  4. Department of Pediatrics, Mie University Graduate School of Medicine, Mie, Japan

    Hidemi Toyoda & Yoshihiro Komada

  5. Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Mie, Japan

    Eiji Ishikawa, Shinsuke Nomura & Masaaki Ito

Authors
  1. Naomi Ito-Habe
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  2. Hideo Wada
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  3. Takeshi Matsumoto
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  4. Kohshi Ohishi
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  5. Hidemi Toyoda
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  6. Eiji Ishikawa
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  7. Shinsuke Nomura
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  8. Yoshihiro Komada
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  9. Masaaki Ito
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  10. Tsutomu Nobori
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  11. Naoyuki Katayama
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Corresponding author

Correspondence to Hideo Wada.

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Ito-Habe, N., Wada, H., Matsumoto, T. et al. Elevated Von Willebrand factor propeptide for the diagnosis of thrombotic microangiopathy and for predicting a poor outcome. Int J Hematol 93, 47–52 (2011). https://doi.org/10.1007/s12185-010-0732-4

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  • DOI: https://doi.org/10.1007/s12185-010-0732-4

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