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Prepublished online as a Blood First Edition Paper on August 21, 2003; DOI 10.1182/blood-2003-05-1401.

Submitted May 5, 2003
Accepted August 12, 2003
Primary nasal natural killer cell lymphoma: long-term treatment outcome and relationship with the international prognostic index
Chor-Sang Chim, Shing-Yan Ma, Wing-Yan Au, Carolyn Choy, Albert K Lie, Raymond Liang, Chun-Chung Yau, and Yok-Lam Kwong*
Department of Medicine, University of Hong Kong, Queen Mary Hospital, Hong Kong, Hong Kong
Department of Pathology, University of Hong Kong, Queen Mary Hospital, Hong Kong, Hong Kong
Department of Radiation Oncology, University of Hong Kong, Queen Mary Hospital, Hong Kong, Hong Kong
* Corresponding author; email: ylkwong{at}hkucc.hku.hk.
Nasal natural-killer (NK) cell lymphoma is rare, so that its optimal therapy, long-term outcome and prognostic factors are unclear. Fifty-two male and fifteen female patients with well-characterized nasal NK cell lymphomas were analyzed retrospectively to define the impact of primary therapy on remission and long-term outcome, and the validity of the International-Prognostic-Index (IPI). The majority (84%) had stage I/II disease with IPI 1 (52%). Seven patients received radiotherapy only; 47 patients received anthracycline-containing chemotherapy plus consolidation radiotherapy; 12 patients received non-anthracycline-containing chemotherapy plus radiotherapy. The overall complete-remission (CR) rate was 64.2%; the 20-year overall-survival (OS) and disease-free-survival (DFS) were 37.1% and 33.5%. Frontline radiotherapy was apparently better than chemotherapy for CR (100% versus 59%, p=0.04) and OS (83.3% versus 32.0%, p=0.03). Relapses occurred in four radiotherapy-treated (all local) and 14 chemotherapy-treated patients (nine local, four systemic). Amongst these, five late relapses (four local, one systemic) occurred at 170 (92-348) months from CR. The IPI score was of prognostic significance for the whole group (IPI 1 superior to IPI 2 for 20-year OS: 57.4% versus 27.6%, p=0.012), as well as for patients treated with chemotherapy / radiotherapy (IPI 1 superior to IPI 2 for CR: 76.7% versus 35.7%, p=0.017; and 10-year OS: 63.8% versus 26.8%, p=0.003).

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