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Advances in Hematology
Volume 2010, Article ID 627401, 5 pages
http://dx.doi.org/10.1155/2010/627401
Review Article

Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type

Division of Haematology, Medical Oncology and Bone Marrow Transplantation, Department of Medicine, University of Hong Kong, Queen Mary Hospital, Hong Kong

Received 4 August 2010; Accepted 20 December 2010

Academic Editor: Jorge Enrique Romaguera

Copyright © 2010 Harinder Gill et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The World Health Organization (WHO) classification recognizes 2 main categories of natural killer (NK) cell-derived neoplasms, namely, extranodal NK/T-cell lymphoma, nasal type, and aggressive NK-cell leukaemia. Extranodal nasal NK/T-cell lymphoma is more frequent in the Far East and Latin America. Histopathological and immunophenotypical hallmarks include angiocentricity, angiodestruction, expression of cytoplasmic CD3 epsilon (ε), CD56, and cytotoxic molecules and evidence of Epstein-Barr virus (EBV) infection. Early stage disease, in particular for localized lesion in the nasal region, is treated with chemotherapy and involved-field radiotherapy. On the other hand, multiagent chemotherapy is the mainstay of treatment for advanced or disseminated disease. L-asparaginase-containing regimens have shown promise in treating this condition. The role of autologous hematopoietic stem cell transplantation is yet to be clearly defined. Allogeneic hematopoietic stem cell transplantation, with the putative graft-versus-lymphoma effect, offers a potentially curative option in patients with advanced disease.