Two young adult cases of Epstein-Barr virus associated-hemophagocytic lymphohistiocytosis with monoclonal proliferation of virus-infected cells within a short period after primary infection

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  • IDUTSU Kensaku
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • ABE Yasunobu
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • MATSUSHIMA Takamitsu
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • SADA Eriko
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • OHTSUKA Rie
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • KIYASU Junichi
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • SHIRATSUCHI Motoaki
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • KOTOH Kazuhiro
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • NISHIMURA Junji
    Department of Clinical Immunology, Medical Institute of Bioregulation, Kyushu University
  • OHGA Shouichi
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University
  • TAKAYANAGI Ryoichi
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University

Bibliographic Information

Other Title
  • 初感染後早期にEBウイルス感染細胞のモノクローナルな増殖及び血球貪食症候群を来した若年成人2症例
  • 症例報告 初感染後早期にEBウイルス感染細胞のモノクローナルな増殖及び血球貪食症候群を来した若年成人2症例
  • ショウレイ ホウコク ショカンセンゴ ソウキ ニ EB ウイルス カンセン サイボウ ノ モノクローナルナ ゾウショク オヨビ ケッキュウ ドンショク ショウコウグン オ キタシタ ジャクネン セイジン 2 ショウレイ

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe complication of Epstein-Barr virus (EBV) infection. Interactions between EBV-infected T cells and activated macrophages cause several conditions such as pancytopenia, liver dysfunction and coagulopathy. We describe here two young adults with EBV-associated HLH with monoclonal proliferation of EBV-infected T cells within a short period after infectious mononucleosis as a primary infection. One patient was a 16-year-old man who developed severe pancytopenia and liver dysfunction two months after infectious mononucleosis. Bone marrow examination showed hemophagocytosis, and laboratory data demonstrated monoclonal proliferation of EBV-infected T cells. Several treatments such as immunosuppressive therapy, chemotherapy and hematopoietic stem cell transplantation were not effective, and the patient died of progressive disease. The other patient was a 19-year-old woman who developed thrombocytopenia and liver dysfunction two months after infectious mononucleosis. Findings of hemophagocytosis and monoclonal proliferation of EBV-infected T cells were similar to those in the first case. Clinical signs and symptoms were resolved completely by immunosuppressive therapy containing methyl-prednisolone and cyclosporine. Since these two cases each demonstrated a distinct clinical course, an investigation of the prognostic factors and treatment strategies for EBV-HLH is warranted.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 49 (11), 1541-1547, 2008

    The Japanese Society of Hematology

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