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Massive expansion of EBV+ monoclonal T cells with CD5 down regulation in EBV-associated haemophagocytic lymphohistiocytosis
  1. Ming-Tsan Lin1,2,
  2. Hui-Mei Chang3,
  3. Chang-Jen Huang4,
  4. Woan-Ling Chen1,
  5. Chi-Yung Lin,
  6. Ching-Yang Lin1,5,
  7. Shih-Sung Chuang6
  1. 1Department of Pediatrics, Changhua Christian Hospital, Changhua, Taiwan
  2. 2National Cheng-Kung University Medical School, Tainan, Taiwan
  3. 3Department of Pathology, Changhua Christian Hospital, Changhua, Taiwan
  4. 4Institue of Biological Chemistry, Academia Sinica, Taipei, Taiwan
  5. 5Department of Clinical Pathology, Changhua Christian Hospital, Changhua, Taiwan; Institute of Medical Research, Chang Jung Christian University, Tainan, Taiwan
  6. 6Chi-Mei Medical Centre, Tainan; Taipei Medical University, Taipei, Taiwan
  1. Correspondence to:
    Dr S-S Chuang
    Department of Pathology, Chi-Mei Medical Centre, 901 Chung-Hwa Road, Yung-Kang City, Tainan County 710, Taiwan; cmh5301{at}mail.chimei.org.tw

Abstract

Haemophagocytic lymphohistiocytosis (HLH) comprises primary and secondary forms; the secondary form is most commonly triggered by the Epstein–Barr virus (EBV; EBV-HLH). Patients with EBV-HLH usually exhibit oligoclonal or monoclonal T cell proliferation, which may mimic T cell lymphoproliferative disorder (T-LPD). This article reports on EBV-HLH in a 17-month-old girl with an extreme surge of reactive T lymphocytosis (absolute count 167×109/l) with CD5 down regulation. Bone marrow aspirate and trephine contained florid haemophagocytosis and massive infiltration of CD3+ Epstein–Barr virus-encoded RNA+ lymphocytes, as seen by double labelling. These lymphocytes were monoclonal for EBV and T cell receptor γ chain gene rearrangement. The patient responded dramatically to intravenous immunoglobulin, interferon α2b, ganciclovir and prednisolone, suggesting restoration of her immune system and eradication of the clonal T cells through these immunoregulatory agents. Thus, careful clinicopathological correlation is warranted in the interpretation of immunophenotyping and clonality data in T cell proliferation in association with EBV-HLH to avoid erroneous diagnosis of T-LPD.

  • EBV, Epstein–Barr virus
  • HLH, haemophagocytic lymphohistiocytosis
  • PBMC, peripheral blood mononuclear cell
  • TCR, T cell receptor
  • T-LPD, T cell lymphoproliferative disorder

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Footnotes

  • Competing interests: None declared.

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