Massive expansion of EBV+ monoclonal T-cells with CD5 down regulation in EBV-associated haemophagocytic lymphohistiocytosis
Resource
Journal of Clinical Pathology, 60(1), 101-103
Journal
Journal of Clinical Pathology
Pages
101-103
Date Issued
2007
Date
2007
Author(s)
Lin, Ming-Tsan
Chang, Hui-Mei
Huang, Chang-Jen
Chen, Woan-Ling
Lin, Chi-Yung
Lin, Ching-Yang
Chuang, Shih-Sung
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 167x10(9)/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 gamma chain gene rearrangement. The patient responded dramatically to intravenous immunoglobulin, interferon alpha2b, 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.
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