A Case of Intractable Hemophagocytic Syndrome Associated with Systemic Lupus Erythematosus Resistant to Corticosteroids and Intravenous Cyclophosphamide that Was Successfully Treated with Cyclosporine A

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著者

    • Toko Hirofumi
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Hagiwara Shinya
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Hirota Tomoya
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Kondo Yuya
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Matsumoto Isao
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Sumida Takayuki
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Tsuboi Hiroto
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Umeda Naoto
    • Department of Rheumatology, Tsuchiura Kyodo General Hospital, Japan
    • Honda Fumika
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Ohyama Ayako
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Takahashi Hidenori
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Abe Saori
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Yokosawa Masahiro
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan
    • Asashima Hiromitsu
    • Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Japan

抄録

<p>Hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE), dubbed acute lupus hemophagocytic syndrome (ALHS), is an intractable complication of SLE. A 24-year-old man who had been diagnosed with SLE three months previously, presented with fever, rash, hallucination, and pancytopenia accompanied with hyperferritinemia and bone marrow hemophagocytosis. He was diagnosed with ALHS and neuropsychiatric (NP)-SLE. Although 4 courses of methylprednisolone pulse therapy and 1 course of intravenous cyclophosphamide (IVCY) improved his NP-SLE, his ALHS did not respond. However, the addition of cyclosporine A (CsA) led to a rapid remission from ALHS. This suggests the usefulness of CsA in the treatment of intractable, corticosteroid- and IVCY-resistant ALHS. </p>

収録刊行物

  • Internal Medicine

    Internal Medicine, 2018

    一般社団法人 日本内科学会

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