Atypical hemolytic uremic syndrome in an elderly patient successfully treated with eculizumab

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  • FUKASAWA Chisa
    Department of Hematology/Oncology, University of Yamanashi
  • OOISHI Saori
    Department of Hematology/Oncology, University of Yamanashi
  • KUMAGAI Takuma
    Department of Hematology/Oncology, University of Yamanashi
  • KOSHIISI Megumi
    Department of Hematology/Oncology, University of Yamanashi
  • SUEKI Yuki
    Department of Hematology/Oncology, University of Yamanashi
  • NAKAJIMA Kei
    Department of Hematology/Oncology, University of Yamanashi
  • MITSUMORI Toru
    Department of Hematology/Oncology, University of Yamanashi
  • YOSHIDA Yoko
    Division of Nephrology and Endocrinology, The University of Tokyo Hospital
  • KATO Hideki
    Division of Nephrology and Endocrinology, The University of Tokyo Hospital
  • NANGAKU Masaomi
    Division of Nephrology and Endocrinology, The University of Tokyo Hospital
  • MIYATA Toshiyuki
    Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center
  • KIRITO Keita
    Department of Hematology/Oncology, University of Yamanashi

Bibliographic Information

Other Title
  • Eculizumabが奏効した高齢発症非典型溶血性尿毒症症候群
  • 症例報告 第6回日本血液学会関東甲信越地方会 優秀演題 Eculizumabが奏効した高齢発症非典型溶血性尿毒症症候群
  • ショウレイ ホウコク ダイ6カイ ニホン ケツエキ ガッカイ カントウ コウシンエツチホウカイ ユウシュウ エンダイ Eculizumab ガ ソウコウ シタ コウレイ ハッショウ ヒテンケイ ヨウケツセイ ニョウドクショウ ショウコウグン

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Abstract

<p>Herein, we present an elderly onset case of aHUS successfully treated with eculizumab. An 80-year-old woman with severe anemia, thrombocytopenia, and acute renal dysfunction was admitted to our hospital. A laboratory test revealed steep elevation in the LDH level, and the peripheral blood smear showed erythrocyte fragmentations. Accordingly, we diagnosed thrombotic microangiopathy, and treatment with plasma exchange was immediately initiated. In addition, she required hemodialysis because of rapid impairment of the renal function. After excluding Shiga toxin-producing Escherichia coli infection and malignancy and confirming her ADMTS13 activity above 10%, we diagnosed aHUS, according to the Japanese diagnostic criteria for aHUS. Next, we initiated treatment with eculizumab. Her hematological findings improved 23 days after the starting of eculizumab. In addition, her renal function gradually recovered, and hemodialysis was discontinued. The genetic test for several complement regulatory genes tested negative. The onset of aHUS is reported in children or young adults and is rarely reported in elderly. However, our case suggests the importance of precisely diagnosing aHUS and initiating early administration of eculizumab for improving the outcome even in elderly patients.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 59 (2), 182-186, 2018

    The Japanese Society of Hematology

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