Journal
HEMATOLOGICAL ONCOLOGY
Volume 26, Issue 2, Pages 66-72Publisher
WILEY
DOI: 10.1002/hon.847
Keywords
natural killer cell; azurophilic granule; Epstein-Barr virus; CD56; cytotoxic molecule
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Extranodal NK/T-cell lymphoma, nasal type (ENKL) is mostly endemic to East Asia. It predominantly occurs in the nasal or paranasal areas and less frequently in the skin. Most of the tumours show NK-cell, but rarely T-cell, phenotypes. The Epstein-Barr virus (EBV) genome can be usually detected in lymphoma cells. Geographic localization of ENK-L matches the endemic distribution of EBV, suggesting that EBV plays an important role in lymphomagenesis. Originally, NK-cell and T-cell types were believed to present the same clinicopathologic characteristics, but recent data suggest more aggressive characteristics for the NK-cell phenotype. Although ENKL is sensitive to radiotherapy, it shows a poorer response to chemotherapeutic agents than other lymphomas due to expression of p-glycoprotein. Therefore, new therapeutic approaches must be considered. Several new clinical trials are now being conducted in East Asia. Copyright (C) 2008 John Wiley & Sons, Ltd.
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