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Monoclonal proliferation of Epstein-Barr virus-infected T-cells in a patient with virus-associated haemophagocytic syndrome

  • Infectious Diseases
  • Original Paper
  • Published:
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Abstract

Virus-associated haemophagocytic syndrome (VAHS) is a non-neoplastic, generalized histiocytic proliferation disorder showing marked haemophagocytosis associated with systemic viral infection. We describe the case of a 1-year-old girl with Epstein-Barr virus (EBV)-related VAHS, in whom Southern blot analysis showed monoclonal proliferation of bone marrow cells with the EBV genome; detected with the Xho-1 fragment of the latent infection membrane protein genome. EBV serology showed anti-Epstein-Barr virus nuclear associated antigen (EBNA), anti-viral capsid antigen (VCA)-IgG, anti-VCA-IgA elevation and positive EBNA of Sheep red blood cells (SRBC)-rosette-forming bone marrow cells in the late period of her clinical course, indicative of EBV infection. DNA analysis of her bone marrow cells showed monoclonal rearrangement of the T-cell receptor-β and-ψ chain genes but not of the immunoglobulin heavy chain genes. Those results suggest that EBV may infect T-cells, after which the cells proliferate monoclonally. Repeated administration of epipodophyllotoxin VP-16-213 induced remission, but adrenocortical steroid, vincristine, and cyclophosphamide had no effect on the patient's condition. Ours is a first case report of VAHS showing monoclonal proliferation of EBV-infected T-cells.

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Abbreviations

EBV :

Epstein-Barr virus

VAHS :

virus-associated hemophagocytic syndrome

EBNA :

Epstein-Barr virus nuclear-associated antigen

VCA :

viral capsid antigenSRBC sheep red blood cells

VP16 :

epipodophyllotoxin VP-16-213

FACS :

fluorescence-activated cell sorter

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Authors and Affiliations

  1. Department of Paediatrics, Saitama Medical School, Moroyama, Iruma-gu, 350-04, Saitama, Japan

    T. Noma, K. Kou, I. Yoshizawa & Y. Kawano

  2. Department of Virology, National Children's Hospital, Medical Research Centre, Tokyo, Japan

    T. Miyashita & S. Mizutani

  3. Department of Paediatrics, Tokyo Medical and Dental University, Tokyo, Japan

    J. Yata

Authors
  1. T. Noma
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  2. K. Kou
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  3. I. Yoshizawa
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  4. Y. Kawano
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  5. T. Miyashita
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  6. S. Mizutani
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  7. J. Yata
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Noma, T., Kou, K., Yoshizawa, I. et al. Monoclonal proliferation of Epstein-Barr virus-infected T-cells in a patient with virus-associated haemophagocytic syndrome. Eur J Pediatr 153, 734–738 (1994). https://doi.org/10.1007/BF01954490

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  • DOI: https://doi.org/10.1007/BF01954490

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