Chronic active Epstein-Barr virus infection with marked pericardial effusion successfully treated with allogeneic peripheral blood stem cell transplantation

  • MATSUI Shinichiro
    Department of Hematology, Chiba University Hospital
  • TAKEDA Yusuke
    Department of Hematology, Chiba University Hospital
  • ISSHIKI Yusuke
    Department of Hematology, Chiba University Hospital
  • YAMAZAKI Atsuko
    Department of Hematology, Chiba University Hospital
  • NAKAO Sanshiro
    Department of Hematology, Chiba University Hospital
  • TAKAISHI Koji
    Department of Hematology, Chiba University Hospital
  • NAGAO Yuhei
    Department of Hematology, Chiba University Hospital
  • HASEGAWA Nagisa
    Department of Hematology, Chiba University Hospital
  • TOGASAKI Emi
    Department of Hematology, Chiba University Hospital
  • SHIMIZU Ryoh
    Department of Hematology, Chiba University Hospital
  • KAWAJIRI Chika
    Department of Hematology, Chiba University Hospital
  • SAKAI Shio
    Department of Hematology, Chiba University Hospital Department of Transfusion Medicine and Cell Therapy, Chiba University Hospital
  • MIMURA Naoya
    Department of Hematology, Chiba University Hospital Department of Transfusion Medicine and Cell Therapy, Chiba University Hospital
  • TAKEUCHI Masahiro
    Department of Hematology, Chiba University Hospital
  • OHWADA Chikako
    Department of Hematology, Chiba University Hospital
  • SAKAIDA Emiko
    Department of Hematology, Chiba University Hospital
  • ISEKI Tohru
    Department of Hematology, Chiba University Hospital Department of Transfusion Medicine and Cell Therapy, Chiba University Hospital
  • IMADOME Ken-Ichi
    Division of Advanced Medicine for Virus Infections, National Research Institute for Child Health and Development
  • NAKASEKO Chiaki
    Department of Hematology, Chiba University Hospital

Bibliographic Information

Other Title
  • 著明な心嚢水貯留を主症状として発症し同種末梢血幹細胞移植が奏効した慢性活動性EBウイルス感染症
  • 症例報告 第3回日本血液学会関東甲信越地方会 優秀演題 著明な心嚢水貯留を主症状として発症し同種末梢血幹細胞移植が奏効した慢性活動性EBウイルス感染症
  • ショウレイ ホウコク ダイ3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 チョメイ ナ シンノウスイ チョリュウ オ シュ ショウジョウ ト シテ ハッショウ シ ドウシュ マッショウケツ カンサイボウ イショク ガ ソウコウ シタ マンセイ カツドウセイ EB ウイルス カンセンショウ

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Abstract

A 23-year-old woman presented with a persistent fever and shortness of breath. Computed tomography showed marked pericardial effusion, hepatosplenomegaly, and cervical and mediastinal lymph node swelling. Epstein-Barr virus (EBV) antibody titers were abnormally elevated, and the copy number of EBV-DNA was increased in peripheral blood. Based on these observations, she was diagnosed with chronic active EBV infection (CAEBV). The EBV-infected cells in her peripheral blood were CD4+T lymphocytes. Fever and pericardial effusion improved following treatment with a combination of prednisolone, etoposide, and cyclosporine; however, peripheral blood EBV-DNA levels remained high. The patient underwent allogeneic peripheral blood stem cell transplantation from an EBV-seronegative, HLA-matched sibling donor, with fludarabine and melphalan conditioning. The post-transplantation course was uneventful, except for mild skin acute graft-versus-host disease (grade 2). EBV-DNA became undetectable in peripheral blood 98 days post transplantation. She has since been in good health without disease recurrence. CAEBV is a potentially fatal disease caused by persistent EBV infection of T lymphocytes or natural killer cells, thus requiring prompt treatment and allogeneic transplantation. Pericardial effusion is rarely observed in CAEBV and can impede its diagnosis. Therefore, we should be aware that patients may present with marked pericardial effusion as an initial manifestation of CAEBV.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 57 (5), 624-629, 2016

    The Japanese Society of Hematology

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