演題抄録

International Symposium

開催概要
開催回
第51回・2013年・京都
 

Extranodal NK/T-cell lymphoma, nasal type (ENKTL)

演題番号 : IS3-7

[筆頭演者]
Won Seog Kim:1 

1:Hematology-Oncology, Samsung Medical Center, Korea

 

Extranodal NK/T-cell lymphoma (ENKTL) is an unique histopathologic subtype, which is strongly associated Epstein−Barr virus (EBV) infection. This disease has skewed geographic distribution, more common in Asia and Central-South America. Unlike other aggressive lymphomas, around 80% of the cases are localized to upper airway region. The prognosis is generally very poor with 30-40% long term survival. Anthracycline-based chemotherapies (CHOP or CHOP-like) give very disappointing response, resulting in 30%-40% long them survival, even in stage I or II cases, because of high expression of p-glycoprotein. Instead, poly-chemotherapies with non-MDR affected agents and L-asparaginase combination are giving much improved outcome. C concurrent chemo-radiation or sequential chemo-radiation with such chemo-regimens are achieving 70-80% of long term survival higher efficacy in localized ENKTL. For advanced disease, L-asparaginased based poly-chemotherapy regimens are achieving promising results. Recently, some cytogenetic and genetic changes have been reported. Deletion of 6q has been most widely reported. Some genetic changes of tumor suppressor genesand oncogenes have been reported, including HACE1, PRDM1, FOXO3, P53, PDGFR , PLK1, Aurora-A, JAK3 and so on. Based on the knowledge on the genetic alteration, novel agent trial should be valuable in a near future.

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