%0 Journal Article
%T Aggressive Mature Natural Killer Cell Neoplasms: From EBV-Infection to Disease Etiopathogeny
%A Margarida Lima
%J Open Journal of Immunology
%D 2014
%R 10.4172/2155-9864.1000193
%X Mature Natural Killer (NK) cell neoplasms are rare tumors with higher prevalence in Asia, Central and South America, which are related to Epstein Barr Virus (EBV) infection. Nature killer /T cell lymphoma, nasal type, presents as a localized or generalized destructive tumor, affecting the nose, the upper aero digestive tract or any organ or tissue, whereas aggressive NK-cell leukemia manifests as a systemic disease that preferentially affects the bone marrow, the spleen and the liver, and rapidly evolve to multiorgan failure resulting in death. Both NK-cell neoplasms arise as a consequence of the inability of the immune system to control EBV infection and of the transforming potential of multiple EBV gene products. Chronic active EBV infection and EBV-related lymphoproliferative disorders of NK-cells are predisposing conditions. The tumor NK-cells do express an EBV infection type II latency pattern, specific EBV-encoded latent membrane proteins and early region EBV RNAs being detected on lymphoma cells. The EBV encoded proteins and non-coding EBV RNAs and micro-RNAs expressed on the infected cells are involved in immune deregulation and play a crucial role in cell transformation and oncogenesis. This review addresses the mechanisms used by EBV to infect the cells and to evade the immune-surveillance as well as to induce cell survival and transformation, and characterizes the spectrum of the clinical manifestations associated with chronic EBV-infection and related T-and NK-lymphoproliferative disorders. Improving the knowledge in this subject will help to develop new therapeutic approaches for chronic EBV-infection and even prevention strategies for the aggressive NK-cell malignancies
%K Margarida Lima
%K NK-cell neoplasms
%K NK/T-cell lymphoma
%K Nasal-type
%K Aggressive NK-cell leukemia
%K Epstein Barr Virus
%K Chronic active EBVinfection
%K Viral-induced cell transformation
%U https://www.longdom.org/abstract/aggressive-mature-natural-killer-cell-neoplasms-from-ebvinfection-to-disease-etiopathogeny-27974.html