Clinical features of adult-onset chronic active Epstein-Barr virus infection

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  • KAWAMOTO Keisuke
    Department of Hematology, Endocrinology, and Metabolism, Faculty of Medicine, Niigata University Department of Pathology, Kurume University School of Medicine
  • MIYOSHI Hiroaki
    Department of Pathology, Kurume University School of Medicine
  • SETO Masao
    Department of Pathology, Kurume University School of Medicine
  • KIMURA Hiroshi
    Department of Virology, Faculty of Medicine, Nagoya University
  • OHSHIMA Koichi
    Department of Pathology, Kurume University School of Medicine

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Other Title
  • 成人発症の慢性活動性EBウイルス感染症の臨床的特徴
  • セイジン ハッショウ ノ マンセイ カツドウセイ EB ウイルス カンセンショウ ノ リンショウテキ トクチョウ

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Abstract

<p>Chronic active Epstein-Barr virus infection (CAEBV) presents with mononucleosis-like symptoms such as chronic persistent or recurrent pyrexia, lymphadenopathy, and hepatosplenomegaly because of the reactivation of Epstein-Barr virus (EBV) as demonstrated by the recurrence of EBV-infected cells. The mechanism of CAEBV remains obscure, and CAEBV can lead to fatal conditions such as hemophagocytic syndrome and malignant lymphoma by clonal expansion of EBV-infected T- or NK-cells. Without hematopoietic stem cell transplantation, CAEBV has a poor prognosis. CAEBV is listed in the revised 2016 World Health Organization classification as a chronic active EBV infection of T- and NK-cell types, systemic form, among EBV-positive T- and NK-cell lymphoproliferative diseases of childhood. However, similar clinical conditions have been reported in adult patients. Therefore, we investigated the clinical features of adult patients with CAEBV-like features (adult-onset CAEBV) in a relatively small number of cases. Additionally, genetic alterations related to CAEBV development have also been reported. Along with these results, we reviewed the clinical characteristics of adult-onset CAEBV.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 60 (8), 944-952, 2019

    The Japanese Society of Hematology

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