Therapeutic modality of 11 patients with TTP in a single institution in Miyazaki from 2000 to 2011

Noriaki Kawano, Naoko Yokota-Ikeda, Shuro Yoshida, Takuro Kuriyama, Kiyoshi Yamashita, Yasuhiro Sugio, Shigeyoshi Makino, Nobuyuki Ono, Yasushi Inoue, Daisuke Himeji, Kieko Kodama, Shigehiro Uezono, Yoshiya Shimao, Akira Ueda, Masanori Matsumoto, Hisayo Iino, Yoshihiro Fujimura

    Research output: Contribution to journalArticle

    4 Citations (Scopus)

    Abstract

    Objective Thrombotic thrombocytopenic purpura (TTP) is a life-threatening generalized disease with pathological features that are termed thrombotic microangiopathies. Since the discovery of the von Willebrand factor-cleaving protease [a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)], it is widely known that approximately two-thirds of TTP patients have a severe deficiency of ADAMTS13 activity due to gene mutations or acquired autoantibodies to this enzyme. However, the remaining one-third of TTP patients have only moderately reduced or almost normal ADAMTS13 activity. To elucidate the clinical characteristics and outcomes of these two types of TTP, we have retrospectively analyzed the cases of acquired TTP patients treated in a single institution from 2000 to 2011. Methods Our case studies include 11 TTP patients, of which 5 were considered idiopathic and 6 had cases of TTP associated with underlying diseases such as non-Hodgkin lymphoma or connective tissue diseases. Results These patients were treated with a combination therapy of plasma exchange and steroids and with several adjunctive therapeutic regimens including the on-label use of cyclophosphamide and cyclosporine and the off-label use of high-dose steroid or immunoglobulin with rituximab. Splenectomies were not performed. As a result of these treatments, 6 out of the 7 patients with ADAMTS13 activity deficient TTP achieved a complete remission without relapse, but the remaining 4 patients with non-ADAMTS13 activity deficient TTP all died without complete remission. Conclusion We present herein the detailed clinical courses of 11 patients with TTP and address our experiences with the efficacy of various therapeutic regimens. This case-oriented study should be helpful to the physicians who directly care for TTP patients, and may provide a future direction for developing a more efficient treatment modality.

    Original languageEnglish
    Pages (from-to)1883-1891
    Number of pages9
    JournalInternal Medicine
    Volume52
    Issue number17
    DOIs
    Publication statusPublished - Sep 9 2013

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    Thrombotic Thrombocytopenic Purpura
    Therapeutics
    Steroids
    Thrombotic Microangiopathies
    Off-Label Use
    Thrombospondin 1
    Disintegrins
    Plasma Exchange
    Connective Tissue Diseases
    Metalloproteases
    Splenectomy
    Autoantibodies
    Non-Hodgkin's Lymphoma
    Cyclophosphamide
    Cyclosporine
    Immunoglobulins
    Physicians

    All Science Journal Classification (ASJC) codes

    • Internal Medicine

    Cite this

    Kawano, N., Yokota-Ikeda, N., Yoshida, S., Kuriyama, T., Yamashita, K., Sugio, Y., ... Fujimura, Y. (2013). Therapeutic modality of 11 patients with TTP in a single institution in Miyazaki from 2000 to 2011. Internal Medicine, 52(17), 1883-1891. https://doi.org/10.2169/internalmedicine.52.8253

    Therapeutic modality of 11 patients with TTP in a single institution in Miyazaki from 2000 to 2011. / Kawano, Noriaki; Yokota-Ikeda, Naoko; Yoshida, Shuro; Kuriyama, Takuro; Yamashita, Kiyoshi; Sugio, Yasuhiro; Makino, Shigeyoshi; Ono, Nobuyuki; Inoue, Yasushi; Himeji, Daisuke; Kodama, Kieko; Uezono, Shigehiro; Shimao, Yoshiya; Ueda, Akira; Matsumoto, Masanori; Iino, Hisayo; Fujimura, Yoshihiro.

    In: Internal Medicine, Vol. 52, No. 17, 09.09.2013, p. 1883-1891.

    Research output: Contribution to journalArticle

    Kawano, N, Yokota-Ikeda, N, Yoshida, S, Kuriyama, T, Yamashita, K, Sugio, Y, Makino, S, Ono, N, Inoue, Y, Himeji, D, Kodama, K, Uezono, S, Shimao, Y, Ueda, A, Matsumoto, M, Iino, H & Fujimura, Y 2013, 'Therapeutic modality of 11 patients with TTP in a single institution in Miyazaki from 2000 to 2011', Internal Medicine, vol. 52, no. 17, pp. 1883-1891. https://doi.org/10.2169/internalmedicine.52.8253
    Kawano N, Yokota-Ikeda N, Yoshida S, Kuriyama T, Yamashita K, Sugio Y et al. Therapeutic modality of 11 patients with TTP in a single institution in Miyazaki from 2000 to 2011. Internal Medicine. 2013 Sep 9;52(17):1883-1891. https://doi.org/10.2169/internalmedicine.52.8253
    Kawano, Noriaki ; Yokota-Ikeda, Naoko ; Yoshida, Shuro ; Kuriyama, Takuro ; Yamashita, Kiyoshi ; Sugio, Yasuhiro ; Makino, Shigeyoshi ; Ono, Nobuyuki ; Inoue, Yasushi ; Himeji, Daisuke ; Kodama, Kieko ; Uezono, Shigehiro ; Shimao, Yoshiya ; Ueda, Akira ; Matsumoto, Masanori ; Iino, Hisayo ; Fujimura, Yoshihiro. / Therapeutic modality of 11 patients with TTP in a single institution in Miyazaki from 2000 to 2011. In: Internal Medicine. 2013 ; Vol. 52, No. 17. pp. 1883-1891.
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    abstract = "Objective Thrombotic thrombocytopenic purpura (TTP) is a life-threatening generalized disease with pathological features that are termed thrombotic microangiopathies. Since the discovery of the von Willebrand factor-cleaving protease [a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)], it is widely known that approximately two-thirds of TTP patients have a severe deficiency of ADAMTS13 activity due to gene mutations or acquired autoantibodies to this enzyme. However, the remaining one-third of TTP patients have only moderately reduced or almost normal ADAMTS13 activity. To elucidate the clinical characteristics and outcomes of these two types of TTP, we have retrospectively analyzed the cases of acquired TTP patients treated in a single institution from 2000 to 2011. Methods Our case studies include 11 TTP patients, of which 5 were considered idiopathic and 6 had cases of TTP associated with underlying diseases such as non-Hodgkin lymphoma or connective tissue diseases. Results These patients were treated with a combination therapy of plasma exchange and steroids and with several adjunctive therapeutic regimens including the on-label use of cyclophosphamide and cyclosporine and the off-label use of high-dose steroid or immunoglobulin with rituximab. Splenectomies were not performed. As a result of these treatments, 6 out of the 7 patients with ADAMTS13 activity deficient TTP achieved a complete remission without relapse, but the remaining 4 patients with non-ADAMTS13 activity deficient TTP all died without complete remission. Conclusion We present herein the detailed clinical courses of 11 patients with TTP and address our experiences with the efficacy of various therapeutic regimens. This case-oriented study should be helpful to the physicians who directly care for TTP patients, and may provide a future direction for developing a more efficient treatment modality.",
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    T1 - Therapeutic modality of 11 patients with TTP in a single institution in Miyazaki from 2000 to 2011

    AU - Kawano, Noriaki

    AU - Yokota-Ikeda, Naoko

    AU - Yoshida, Shuro

    AU - Kuriyama, Takuro

    AU - Yamashita, Kiyoshi

    AU - Sugio, Yasuhiro

    AU - Makino, Shigeyoshi

    AU - Ono, Nobuyuki

    AU - Inoue, Yasushi

    AU - Himeji, Daisuke

    AU - Kodama, Kieko

    AU - Uezono, Shigehiro

    AU - Shimao, Yoshiya

    AU - Ueda, Akira

    AU - Matsumoto, Masanori

    AU - Iino, Hisayo

    AU - Fujimura, Yoshihiro

    PY - 2013/9/9

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    N2 - Objective Thrombotic thrombocytopenic purpura (TTP) is a life-threatening generalized disease with pathological features that are termed thrombotic microangiopathies. Since the discovery of the von Willebrand factor-cleaving protease [a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)], it is widely known that approximately two-thirds of TTP patients have a severe deficiency of ADAMTS13 activity due to gene mutations or acquired autoantibodies to this enzyme. However, the remaining one-third of TTP patients have only moderately reduced or almost normal ADAMTS13 activity. To elucidate the clinical characteristics and outcomes of these two types of TTP, we have retrospectively analyzed the cases of acquired TTP patients treated in a single institution from 2000 to 2011. Methods Our case studies include 11 TTP patients, of which 5 were considered idiopathic and 6 had cases of TTP associated with underlying diseases such as non-Hodgkin lymphoma or connective tissue diseases. Results These patients were treated with a combination therapy of plasma exchange and steroids and with several adjunctive therapeutic regimens including the on-label use of cyclophosphamide and cyclosporine and the off-label use of high-dose steroid or immunoglobulin with rituximab. Splenectomies were not performed. As a result of these treatments, 6 out of the 7 patients with ADAMTS13 activity deficient TTP achieved a complete remission without relapse, but the remaining 4 patients with non-ADAMTS13 activity deficient TTP all died without complete remission. Conclusion We present herein the detailed clinical courses of 11 patients with TTP and address our experiences with the efficacy of various therapeutic regimens. This case-oriented study should be helpful to the physicians who directly care for TTP patients, and may provide a future direction for developing a more efficient treatment modality.

    AB - Objective Thrombotic thrombocytopenic purpura (TTP) is a life-threatening generalized disease with pathological features that are termed thrombotic microangiopathies. Since the discovery of the von Willebrand factor-cleaving protease [a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)], it is widely known that approximately two-thirds of TTP patients have a severe deficiency of ADAMTS13 activity due to gene mutations or acquired autoantibodies to this enzyme. However, the remaining one-third of TTP patients have only moderately reduced or almost normal ADAMTS13 activity. To elucidate the clinical characteristics and outcomes of these two types of TTP, we have retrospectively analyzed the cases of acquired TTP patients treated in a single institution from 2000 to 2011. Methods Our case studies include 11 TTP patients, of which 5 were considered idiopathic and 6 had cases of TTP associated with underlying diseases such as non-Hodgkin lymphoma or connective tissue diseases. Results These patients were treated with a combination therapy of plasma exchange and steroids and with several adjunctive therapeutic regimens including the on-label use of cyclophosphamide and cyclosporine and the off-label use of high-dose steroid or immunoglobulin with rituximab. Splenectomies were not performed. As a result of these treatments, 6 out of the 7 patients with ADAMTS13 activity deficient TTP achieved a complete remission without relapse, but the remaining 4 patients with non-ADAMTS13 activity deficient TTP all died without complete remission. Conclusion We present herein the detailed clinical courses of 11 patients with TTP and address our experiences with the efficacy of various therapeutic regimens. This case-oriented study should be helpful to the physicians who directly care for TTP patients, and may provide a future direction for developing a more efficient treatment modality.

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