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Blood, 23 April 2009, Vol. 113, No. 17, pp. 3931-3937.
Prepublished online as a Blood First Edition Paper on November 24, 2008; DOI 10.1182/blood-2008-10-185256.


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Submitted October 24, 2008
Accepted November 15, 2008

Clinical differences between nasal and extranasal NK/T-cell lymphoma: a study of 136 cases from the International Peripheral T-cell Lymphoma Project

Wing-yan Au, Dennis D. Weisenburger, Tanin Intragumtornchai, Shigeo Nakamura, Won-Seog Kim, Ivy Sng, Julie Vose, James O. Armitage, and Raymond Liang*

Department of Medicine, Queen Mary Hospital, Hong Kong, China
Departments of Internal Medicine and Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE, United States
Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
Department of Pathology and Clinical Laboratories, Nagoya University Hospital, Nagoya, Japan
Division of Hemato-Oncology, Department of Medicine, Samsung Medical Center, Sunkyunkwan University School of Medicine, Seoul, Korea, Republic of
Department of Pathology, Singapore General Hospital, Singapore, Singapore

* Corresponding author; email: rliang{at}hkucc.hku.hk.

Among 1153 new adult cases of extranodal NK/T-cell lymphoma from 1990-2002 at 22 centers in 13 countries, 136 cases (11.8%) of extranodal NK/T-cell lymphoma were identified (nasal 68%, extranasal 26%, aggressive/unclassifiable 6%). The disease frequency was higher in Asian than in Western countries and in Continental Asia than in Japan. There were no differences in age, gender, ethnicity or immunophenotypic profile between the nasal and extranasal cases, but the latter had more adverse clinical features. The median overall survival (OS) was better in nasal compared to the extranasal cases in early (2.96 vs. 0.36 yrs, p<0.001) and late stage disease (0.8 vs. 0.28 yrs, p=0.031). The addition of radiotherapy for early stage nasal cases yielded survival benefit (p=0.045). Among nasal cases, both the International Prognostic Index (p=0.0057) and Korean NK/T-cell Prognostic Index (p<0.001) were prognostic. In addition, Ki67 proliferation >50%, transformed tumor cells >40%, elevated CRP, anemia (<11g/dl) and thrombocytopenia (<150x109/l) predicts poorer OS for nasal disease. No histological or clinical feature was predictive in extranasal disease. We conclude that the clinical features and treatment response of extranasal NK/T-cell lymphoma are different from of those of nasal lymphoma. However, the underlying features responsible for these differences remain to be defined.


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