Successful treatment with recombinant thrombomodulin for disseminated intravascular coagulation complicated with hemophagocytic syndrome

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  • YAMAZAKI Hiroyuki
    Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University
  • KONDO Tadakazu
    Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University
  • TATSUMI Goichi
    Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University
  • KOTANI Shin-ichi
    Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University
  • ARAI Yasuyuki
    Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University
  • SHIRAKAWA Kotaro
    Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University
  • KITANO Toshiyuki
    Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University
  • HISHIZAWA Masakatsu
    Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University
  • KADOWAKI Norimitsu
    Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University
  • TAKAORI-KONDO Akifumi
    Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University

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Other Title
  • 遺伝子組み換え型ヒトトロンボモジュリン製剤が有効であった血球貪食症候群併発播種性血管内凝固
  • 症例報告 遺伝子組み換え型ヒトトロンボモジュリン製剤が有効であった血球貪食症候群併発播種性血管内凝固
  • ショウレイ ホウコク イデンシ クミカエカタ ヒトトロンボモジュリン セイザイ ガ ユウコウ デ アッタ ケッキュウドンショク ショウコウグン ヘイハツ ハシュセイ ケッカン ナイ ギョウコ

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Description

Recombinant human thrombomodulin (rTM) improves the blood coagulation disorder characteristic of disseminated intravascular coagulation (DIC) as well as, or even better than, other anti-DIC drugs. On post-marketing surveillance, its effectiveness has been recognized for hematologic disorders, sepsis and solid tumor subgroups. However, the effect on hemophagocytic syndrome (HPS) complicated by DIC remains unclear. We treated three HPS patients with rTM in addition to chemotherapy for the underlying diseases including nasal NK/T cell lymphoma, angioimmunoblastic T-cell lymphoma and refractory acute myeloid leukemia post cord blood transplantation. Although being refractory to medical management was suspected in our cases, clinical status rapidly came under control including not only amelioration of the blood coagulation disorder but also inflammatory reactions, such as serum ferritin and lactic acid dehydrogenase abnormalities, which represent HPS activity. These observations suggest that rTM might exert marked synergistic effects on HPS with DIC. Given the results obtained in these three cases, administration of rTM appears to offer a promising method of treating HPS complicated by DIC.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 56 (3), 312-316, 2015

    The Japanese Society of Hematology

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