Please use this identifier to cite or link to this item: https://doi.org/10.1186/s13023-014-0165-x
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
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