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Original Article
- EBV-Positive T/NK-Cell Lymphoproliferative Disease of Childhood
- Mineui Hong, Young Hyeh Ko, Keon Hee Yoo, Hong Hoe Koo, Seok Jin Kim, Won Seog Kim, Heejung Park
- Korean J Pathol. 2013;47(2):137-147. Published online April 24, 2013
- DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.2.137
- 13,567 View
- 113 Download
- 30 Crossref
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Abstract
PDF 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.
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Citations
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Case Report
- Graft-Versus-Host Disease of the Lung after Allogeneic Hematopoietic Stem Cell Transplantation: A Report of Two Cases.
- Ji Hyeon Roh, Joungho Han, Keon Hee Yoo, Kang Mo Ahn, Jihye Kim
- Korean J Pathol. 2009;43(4):378-381.
- DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.4.378
- 3,023 View
- 67 Download
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Abstract
PDF
- Herein, we describe cases of pulmonary acute graft-versus-host disease (aGVHD) in two patients occurring after allogeneic hematopoietic stem cell transplantation (HSCT) due to precursor B-cell acute lymphoblastic leukemia in a 6-year-old patient and in acute myeloid leukemia in a 14-year-old boy. In both cases, chest CT revealed confluent ground-glass attenuation along the bronchovascular bundles, as well as some bronchial dilatation. Microscopically, in both cases we noted a characteristic bronchiolocentric pattern and bronchiolar epithelial changes, which included denudation of the bronchiolar epithelium, regenerating atypical cells, and wall thickening with subepithelial or transmural fibroblast proliferation, along with some lymphocytic infiltration. One patient died on day 86 after allogeneic HSCT due to sudden acute respiratory distress syndrome, and the other patient currently remains alive without active aGVHD. The authors' experiences in these two cases demonstrate that intense awareness of the pathologic findings of GVHD is mandatory after allogeneic HSCT.
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