EBV-Positive T/NK-Cell Lymphoproliferative Disease of Childhood
홍민의, 고영혜, 유건희, 구홍회, 김석진, 김원석, 박희정,
소속 상세정보
홍민의 ( Hong Min-Eui )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
고영혜 ( Ko Young-Hyeh )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
유건희 ( Yoo Keon-Hee )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pediatrics
구홍회 ( Koo Hong-Hoe )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pediatrics
김석진 ( Kim Seok-Jin )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Hematology-Oncology
김원석 ( Kim Won-Seog )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Hematology-Oncology
박희정 ( Park Hee-Jung )
Ewha Womans University Mokdong Hospital Department of Pathology
KMID : 0357920130470020137
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.
키워드
Epstein-Barr virus infections; Lymphoma; T-cell; Killer cells; natural; Lymphoproliferative disorders; Clonality
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