Young adult onset systemic Epstein-Barr virus-positive T-cell lymphoproliferative disorders of childhood

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  • SAKAI Mizu
    Department of Hematology and Respiratory Medicine, Kochi Medical School, Kochi University
  • TOGITANI Kazuto
    Department of Hematology and Respiratory Medicine, Kochi Medical School, Kochi University
  • TSUKUDA Tsukie
    Department of Hematology and Respiratory Medicine, Kochi Medical School, Kochi University
  • ARAKAWA Yu
    Department of Hematology and Respiratory Medicine, Kochi Medical School, Kochi University
  • IKEZOE Takayuki
    Department of Hematology and Respiratory Medicine, Kochi Medical School, Kochi University
  • HIROI Makoto
    Laboratory of Diagnostic Pathology, Kochi Medical School Hospital
  • YOKOYAMA Akihito
    Department of Hematology and Respiratory Medicine, Kochi Medical School, Kochi University

Bibliographic Information

Other Title
  • 若年成人に発症した全身性EBV陽性Tリンパ増殖症小児型
  • 症例報告 若年成人に発症した全身性EBV陽性Tリンパ増殖症小児型
  • ショウレイ ホウコク ジャクネン セイジン ニ ハッショウ シタ ゼンシンセイ EBV ヨウセイ Tリンパ ゾウショクショウ ショウニガタ

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Description

A 20-year-old woman had a fever, pancytopenia, and liver failure, and was suspected to be suffering from chronic active Epstein-Barr virus (EBV) infection, based on the detection of high EBV-DNA and EBV antibody titers at another hospital. At our institution one month later, clinical manifestations had diminished, and antibody titers had decreased but remained elevated relative to normal levels. Four days later, the patient required hospitalization due to fever, liver damage, and cervical lymphadenopathy. Bone marrow examination and lymph node biopsy results showed EBV-positive cytotoxic T-cells that were predominantly CD4-positive. The disease followed a fulminant course and the patient died of multiple organ failure on hospitalization day 11. Because complicated chromosomal aberrations and T-cell receptor gene rearrangements were identified, we diagnosed her as having systemic EBV-positive T-cell lymphoproliferative disorder of childhood. This disease type includes a lymphoproliferative disorder that is associated with chronic active EBV infection. However, it is clinically different from the type following acute EBV infection. We consider distinguishing between these two types to be important for selecting an early diagnostic procedure and the optimal therapy.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 56 (5), 501-505, 2015

    The Japanese Society of Hematology

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