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 Prepublished online as a Blood First Edition Paper on June 19, 2003; DOI 10.1182/blood-2003-01-0295.

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Submitted February 6, 2003
Accepted June 5, 2003

CD94 transcripts imply a better prognosis in nasal-type extranodal NK/T-cell lymphoma

Chung-Wu Lin, Yu-Hua Chen, Yi-Chun Chuang, Ting-Yun Liu, and Su-Ming Hsu*

Pathology, National Taiwan University Hospital, Taipei, Taiwan

* Corresponding author; email: smhsu2003{at}yahoo.com.

Transcription of NK-cell antigen receptors (NKRs), such as CD94, NKG2, and killer im-munoglobulin-like receptors (KIRs), is developmentally regulated and clonally distributed. We have shown a restricted KIR repertoire (rKIR-R) without monoclonal T-cell receptor rearrangement (mTCR-R) supports an NK-lineage in nasal-type extranodal NK/T-cell lymphoma (NTENL), but does not correlate with clinical outcomes. Developing NK cells express first CD94, then NKG2A, NKG2E, and finally NKG2C. This sequence suggests an immature CD94- and a mature CD94+ subtype of NTENL. Using a rKIR-R without a mTCR-R as a criterion in 25 NTENLs, we confirmed a true NK lineage in 19 cases, including 10 CD94+ and 9 CD94- patients by RT-PCR. Eight of the 10 CD94+ patients but only 2 of the 9 CD94- patients survived beyond 1 year (median survival: 60 months vs 10 months by Meier-Kaplan survival analysis, p=0.026 by Cox-F test). The remaining 6 patients had a rKIR-R plus a mTCR-R, suggesting mixed NK/T differentiation. They were CD94- by RT-PCR, found predominantly in young females, and had a median survival of 35 months. Thus, based on the transcripts of NKRs, a division of NTENLs into CD94+, CD94-, and mixed NK/T types reflects a true biological divergence with different clinical behaviors.


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C. Bossard, K. Belhadj, F. Reyes, N. Martin-Garcia, F. Berger, J. A. Kummer, J. Briere, A.-C. Baglin, S. Cheze, J. Bosq, et al.
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