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Title: | Prognostic implication of morphology, cyclinE2 and proliferation in EBV-associated T/NK lymphoproliferative disease in non-immunocompromised hosts | Authors: | Ng, S.-B Ohshima, K Selvarajan, V Huang, G Choo, S.-N Miyoshi, H Wang, S Chua, H.-C Yeoh, A.E.-J Quah, T.-C Koh, L.-P Tan, P.-L Chng, W.-J |
Keywords: | CCNE2 protein, human cycline Ki 67 antigen adolescent adult cell proliferation child Epstein Barr virus Epstein Barr virus infection female gene expression profiling genetics human immunocompromised patient immunohistochemistry infant isolation and purification Japan lymphoproliferative disease male metabolism middle aged natural killer cell pathology preschool child prognosis Singapore survival rate T lymphocyte virology young adult Adolescent Adult Cell Proliferation Child Child, Preschool Cyclins Epstein-Barr Virus Infections Female Gene Expression Profiling Herpesvirus 4, Human Humans Immunocompromised Host Immunohistochemistry Infant Japan Ki-67 Antigen Killer Cells, Natural Lymphoproliferative Disorders Male Middle Aged Prognosis Singapore Survival Rate T-Lymphocytes Young Adult |
Issue Date: | 2014 | Citation: | Ng, S.-B, Ohshima, K, Selvarajan, V, Huang, G, Choo, S.-N, Miyoshi, H, Wang, S, Chua, H.-C, Yeoh, A.E.-J, Quah, T.-C, Koh, L.-P, Tan, P.-L, Chng, W.-J (2014). Prognostic implication of morphology, cyclinE2 and proliferation in EBV-associated T/NK lymphoproliferative disease in non-immunocompromised hosts. Orphanet journal of rare diseases 9 : 165. ScholarBank@NUS Repository. https://doi.org/10.1186/s13023-014-0165-x | Abstract: | BACKGROUND: EBV-associated T/NK-cell lymphoproliferative diseases (TNKLPD) is a rare spectrum of disease that occurs more commonly in Asia, and Central and South America. It commonly affects children and young adults and is an aggressive disease that is poorly understood with no known biologic markers that can predict prognosis. The systemic form of TNKLPD includes chronic active EBV infection of T/NK type, aggressive NK cell leukemia and systemic EBV?+?T-cell lymphoproliferative disease of childhood.METHODS: In this study, we analyse the clinicopathologic and genetic features of 22 cases of systemic TNKLPD in non-immunocompromised patients, including chronic active EBV infection of T/NK cell type and systemic EBV?+?T-cell lymphoproliferative disease of childhood. We also performed gene expression profiling in a subset of cases to identify markers that may be of prognostic relevance and validated our results using immunohistochemistry.RESULTS: The median age is 14.9 years and two of our 22 cases occurring in patients older than 30 years. Fifteen of 17 cases (88%) with adequate data were of T-cell origin. Eleven of 22 cases revealed polymorphic cellular infiltrate (P-group) while the rest showed monomorphic lymphoid infiltrate (M-group). We found a significant difference in survival between P-group vs M-group patients with median survival not yet reached in P-group, and 1 month in M-group (p = 0.0001), suggesting a role for morphology in predicting patient outcome. We also performed gene expression profiling in a subset of patients and compared the genes differentially expressed between P-group and M-group cases to identify markers of prognostic value. We identified cyclin E2 gene and protein to be differentially expressed between patients with good outcome (P-group, median expression 8%) and poor outcome (M-group, median expression 42%) (p = 0.0005). In addition, the upregulation of cyclin E2 protein in M-group cases correlated with a higher Ki67 proliferation rate (Pearson correlation r = 0.73, p = 0.0006) detected by immunohistochemistry. High cyclin E2 expression was also significantly associated with shorter survival (p = 0.0002).CONCLUSION: Our data suggests the potential role of monomorphic morphology, high cyclin E2 and Ki67 expression as adverse prognostic factors for TNKLPD. | Source Title: | Orphanet journal of rare diseases | URI: | https://scholarbank.nus.edu.sg/handle/10635/174151 | ISSN: | 17501172 | DOI: | 10.1186/s13023-014-0165-x |
Appears in Collections: | Elements Staff Publications |
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