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High Titer of ADAMTS13 Inhibitor Associated with Thrombotic Microangiopathy of the Gut and Skeletal Muscle after Allogeneic Hematopoietic Stem Cell Transplantation

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Abstract

Transplantation-associated thrombotic microangiopathy (TMA) is one of the main complications after hematopoietic stem cell transplantation (HSCT). At the time of onset of gut TMA, a patient developed a high titer of an inhibitor of the non—immunoglobulin G type to ADAMTS13, which physiologically hydrolyzes von Willebrand factor to control spontaneous intravascular thrombus formation.The patient developed symptoms of myositis, a disorder that has occasionally been reported to manifest after HSCT and to resemble some idiopathic autoimmune diseases. However, a muscle biopsy specimen presented pathologic findings of TMA, including microvascular platelet thrombus formation, without inflammatory lymphocyte infiltration. ADAMTS13 activities returned to normal after steroid treatment, and the improvement of TMA symptoms followed. This patient appears to represent a rare case of post-HSCT TMA associated with the development of an ADAMTS13 inhibitor.

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Authors and Affiliations

  1. Department of Hematology, Nagoya University Graduate School of Medicine, 65-Tsurumai-cho, Showa-ku, 466-8560, Nagoya, Japan

    Tatsuya Adachi, Tadashi Matsushita, Kanji Hirashima, Tetsuya Nishida, Makoto Murata, Akira Katsumi & Tomoki Naoe

  2. Departments of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan

    Akira Inukai

  3. Department of Pathology, Nagoya University Hospital, Nagoya, Japan

    Ryoichi Ichihashi

  4. Department of Pathology, Japanese Red Cross Nagoya First Hospital, Nagoya, Japan

    Masafumi Ito

  5. Divisions of Hematology, Nagoya Medical Center, Nagoya, Japan

    Toshiya Yokozawa

  6. Divisions of Cardiology, Nagoya Medical Center, Nagoya, Japan

    Mutsuharu Hayashi

  7. Department of Medical Technology, Nagoya University School of Health Sciences, Nagoya, Japan

    Tetsuhito Kojima

  8. Divisions of Nagoya Medical Center, Nagoya, Japan

    Hidehiko Saito

Authors
  1. Tatsuya Adachi
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  11. Akira Katsumi
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  13. Hidehiko Saito
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  14. Tomoki Naoe
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Correspondence to Tadashi Matsushita.

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Adachi, T., Matsushita, T., Ichihashi, R. et al. High Titer of ADAMTS13 Inhibitor Associated with Thrombotic Microangiopathy of the Gut and Skeletal Muscle after Allogeneic Hematopoietic Stem Cell Transplantation. Int J Hematol 83, 415–419 (2006). https://doi.org/10.1532/IJH97.05157

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  • DOI: https://doi.org/10.1532/IJH97.05157

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