Korean J Pathol.  2013 Apr;47(2):137-147.

EBV-Positive T/NK-Cell Lymphoproliferative Disease of Childhood

• Hong M
• Ko YH
• Yoo KH
• Koo HH
• Kim SJ
• Kim WS
• Park H

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.

Keyword

Epstein-Barr virus infections; Lymphoma, T-cell; Killer cells, natural; Lymphoproliferative disorders; Clonality

MeSH Terms

Epstein-Barr Virus Infections
Gene Rearrangement
Genes, T-Cell Receptor
Herpesvirus 4, Human
Histiocytosis
Humans
Hydroa Vacciniforme
Hypersensitivity
Killer Cells, Natural
Lymphocytes
Lymphocytosis
Lymphohistiocytosis, Hemophagocytic
Lymphoma, T-Cell
Lymphoproliferative Disorders
Receptors, Antigen, T-Cell
T-Lymphocytes
Receptors, Antigen, T-Cell