RT Journal Article
SR Electronic
T1 Massive expansion of EBV+ monoclonal T cells with CD5 down regulation in EBV-associated haemophagocytic lymphohistiocytosis
JF Journal of Clinical Pathology
JO J Clin Pathol
FD BMJ Publishing Group Ltd and Association of Clinical Pathologists
SP 101
OP 103
DO 10.1136/jcp.2005.034371
VO 60
IS 1
A1 Lin, Ming-Tsan
A1 Chang, Hui-Mei
A1 Huang, Chang-Jen
A1 Chen, Woan-Ling
A1 Lin, Chi-Yung
A1 Lin, Ching-Yang
A1 Chuang, Shih-Sung
YR 2007
UL http://jcp.bmj.com/content/60/1/101.abstract
AB 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.