Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis subsequent to rabbit antithymocyte globulin administration and successfully treated with rituximab in a patient with aplastic anemia

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  • NAGATA Hiroaki
    Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital
  • FUJINO Takahiro
    Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine
  • MURAMATSU Ayako
    Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital
  • KAWAJI Yuka
    Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital
  • KURIYAMA Kodai
    Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital
  • OSHIRO Muneo
    Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital
  • HIRAKAWA Yoshiko
    Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital
  • IWAI Toshiki
    Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital
  • UCHIYAMA Hitoji
    Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital

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Other Title
  • ウサギATG投与で発症したEBウイルス関連血球貪食性リンパ組織球症にrituximabが奏効した再生不良性貧血
  • 症例報告 第107回近畿血液学地方会 優秀演題 ウサギATG投与で発症したEBウイルス関連血球貪食性リンパ組織球症にrituximabが奏効した再生不良性貧血
  • ショウレイ ホウコク ダイ107カイ キンキ ケツエキガク チホウカイ ユウシュウ エンダイ ウサギ ATG トウヨ デ ハッショウ シタ EB ウイルス カンレン ケッキュウドンショクセイ リンパ ソシキキュウショウ ニ rituximab ガ ソウコウ シタ サイセイ フリョウセイ ヒンケツ

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Description

<p>Rabbit antithymocyte globulin (ATG) is an effective immunosuppressive therapy for patients with aplastic anemia (AA). However, Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD) is a rare but serious complication of the therapy. An 81-year-old man was diagnosed with severe AA on the occasion of melena. Because cyclosporine monotherapy did not improve his condition, rabbit ATG was additionally administered. Thirty-one days after the administration of rabbit ATG, the patient presented with fever and general malaise. His liver and renal function tests showed rapid decline, and the patient went into shock. Although atypical lymphocytes in the peripheral blood, hepatosplenomegaly, and lymphadenopathy were not detected, the peripheral blood EBV-DNA load and serum ferritin levels were high, and his bone marrow aspiration specimen revealed hemophagocytic findings, leading to a diagnosis of EBV-LPD. He was treated with rituximab and recovered immediately. A total of 480 days have passed since the patient was administered the rabbit ATG, and he remains in AA remission without EBV-LPD relapse. This case suggests that rituximab is an effective therapy for EBV-LPD manifesting as EBV-associated hemophagocytic lymphohistiocytosis and indicates that monitoring the EBV-DNA load contributes to the diagnosis.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 59 (4), 407-413, 2018

    The Japanese Society of Hematology

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