Thrombotic thrombocytopenic purpura

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  • 血栓性血小板減少性紫斑病
  • ケッセンセイ ケッショウバン ゲンショウセイ シハンビョウ

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Abstract

<p>In this article, I mainly review the molecular targeted therapy for thrombotic thrombocytopenic purpura (TTP). TTP is one of thrombotic microangiopathies (TMA), which demonstrate hemolytic anemia with red blood cell destruction and thrombocytopenia. Another TTP, hemolytic uremic anemia (HUS) reveals bloody stood and acute kidney failure. As it is difficult to correctly diagnose TTP based on clinical symptoms alone, a confirmation of whether ADAMTS13 activity is lower than 10% is required. In the past few years, TTP was designated as an intractable disease by law, and the clinical application of ADAMTS13 test and rituximab became labeled in Japan. Currently, we are expecting that recombinant ADAMTS13 and caplacizumab for immune TTP will be applied.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 62 (5), 430-434, 2021

    The Japanese Society of Hematology

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