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A second national questionnaire survey of TMA

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Abstract

A second questionnaire survey of Japanese patients with thrombotic microangiopathy (TMA) was carried out to investigate the frequency, laboratory abnormalities and outcome in 2004 and 2005. The first and second surveys evaluated 397 patients including 19 with familial TMA and 378 with acquired TMA. The patients with acquired TMA included 165 with Escherichia coli O-157 infection-related TMA (O-157 TMA), 70 with ADAMTS13-related TMA (ADAMTS13 TMA) and 38 with other types of TMA (other TMA). The rate of ADAMTS13 TMA was significantly higher in patients with collagen diseases than in patients with all other underlying diseases (p < 0.001). The treatment of acquired TMA included plasma exchange (PE), steroids, antiplatelet agents, and anticoagulants, PE was carried out in 91.4% of patients with ADAMTS13 TMA, 68.4% of patients with other TMA and 12.7% of patients with O-157 TMA. The efficacy of PE and steroid therapy tended to be higher in patients with ADAMTS13 TMA than in those with other TMA. The complete remission rate was the highest and the mortality rate was the lowest in the patients with O-157 TMA. The mortality rate tended to be lower in patients with ADAMTS13 TMA than in those with other TMA. However, not all of the patients in our study were examined for ADAMTS13 at the time that this questionnaire survey was conducted.

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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.

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Authors and Affiliations

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

    Naomi Ito-Habe

  2. Department of Molecular and Laboratory Medicine, Mie University Graduate School of Medicine, Mie, Japan

    Hideo Wada

  3. Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan

    Masanori Matsumoto & Yoshihiro Fujimura

  4. Department of Laboratory Medicine, Keio University School of Medicine, Tokyo, Japan

    Mitsuru Murata

  5. Bureau of Social Welfare and Public Health, Tokyo Metropolitan Government, Tokyo, Japan

    Takashi Izuno

  6. Department of Environmental and Occupational Health, Toho University School of Medicine, Tokyo, Japan

    Minoru Sugita

  7. Faculty of Science and Engineering, Life Science and Medical Bioscience, Waseda University, Tokyo, Japan

    Yasuo Ikeda

Authors
  1. Naomi Ito-Habe
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  2. Hideo Wada
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  3. Masanori Matsumoto
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  4. Yoshihiro Fujimura
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  5. Mitsuru Murata
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  6. Takashi Izuno
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  7. Minoru Sugita
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  8. Yasuo Ikeda
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Correspondence to Hideo Wada.

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Ito-Habe, N., Wada, H., Matsumoto, M. et al. A second national questionnaire survey of TMA. Int J Hematol 92, 68–75 (2010). https://doi.org/10.1007/s12185-010-0599-4

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  • DOI: https://doi.org/10.1007/s12185-010-0599-4

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