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EBV-positive T/NK-cell lymphoproliferative disease of childhood

Title
EBV-positive T/NK-cell lymphoproliferative disease of childhood
Authors
Hong M.Ko Y.H.Yoo K.H.Koo H.H.Kim S.J.Kim W.S.Park H.
Ewha Authors
박희정
Issue Date
2013
Journal Title
Korean Journal of Pathology
ISSN
1738-1843JCR Link
Citation
vol. 47, no. 2, pp. 137 - 147
Indexed
SCOPUS WOS scopus
Abstract
Background: Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH), EBV-positive systemic T-cell lymphoproliferative disease (STLPD) of childhood, and chronic active EBV (CAEBV) infection may develop after primary EBV infection. This study reviewed the clinicopathological spectrum of EBV-associated T- and natural killer (NK)-cell LPD, including STLPD and CAEBV infection, with an analysis of T-cell clonality. Methods: Clinicopathological features of seven patients with EBV-associated HLH or STLPD and 12 patients with CAEBV infection were reviewed. Immunohistochemical staining and a T-cell receptor (TCR) gene rearrangement study were performed. Results: STLPD and EBV-positive HLH showed significantly overlapping clinicopathological findings. One patient with STLPD and one patient with EBV-positive HLH demonstrated moderate to severe atypia of the infiltrating lymphocytes, whereas the remaining patients lacked significant atypia. Twelve patients had CAEBV infection, four of whom suffered mosquito-bite hypersensitivity, five showed NK lymphocytosis, and one suffered hydroa vacciniforme. Infiltrating lymphocytes were predominantly small and devoid of atypia. Hemophagocytic histiocytosis was found in seven of 11 patients. Monoclonality was detected in three (50%) of the six patients with successful TCR gene analysis. Conclusions: EBV-positive HLH and STLPD share similar clinicopathological findings and may constitute a continuous spectrum of acute EBV-associated T- or NK-cell proliferative disorders. The distinction of EBV-positive T-cell LPD from EBV-positive HLH may be difficult during routine diagnoses because of the technical limitations of clonality assessment. © 2013 The Korean Society of Pathologists/The Korean Society for Cytopathology.
DOI
10.4132/KoreanJPathol.2013.47.2.137
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의료원 > 의료원 > Journal papers
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