NK-cell lineage predicts poor survival in primary intestinal NK-cell and T-cell lymphomas

Shih Sung Chuang*, Sheng Tsung Chang, Wen Yu Chuang, Wan Ting Huang, Pin Pen Hsieh, Mei Hua Tsou, Yung Liang Liao, Shu Hui Lin, Yen Chuan Hsieh, Chin Li Lu, Ming Jen Sheu, Hongxiang Liu

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

64 Scopus citations


Most primary intestinal natural killer (NK)-cell and T-cell lymphomas (PINKTL) in the Northern Europe are enteropathy-associated T-cell lymphomas, a complication of celiac disease, which is rare in the East. Primary intestinal NK-cell lymphoma is extremely rare and is poorly characterized. We investigated 30 cases of PINKTL from Taiwan with male: female at 2:1, median age at 55.5, 80% with jejunal/ileal involvement, 77% with perforation, 27% with multicentric tumors, and 67% at stage IE. All 7 cases tested for serum IgA anti-tissue transglutaminase were negative. Only 3 (10%) tumors showed enteropathy. Six (20%) were NK-cell lymphoma and 24 (80%) were T-cell lymphoma. The tumor cells in 21/30 (70%) cases were small to medium sized, which correlated with the coexpression of both CD8 and CD56. All tumors expressed at least 1 cytotoxic marker. All 6 NK-cell lymphomas were negative for βF1, diffusely positive for Epstein-Barr virus-encoded mRNA (EBER), and polyclonal for T-cell receptor gene rearrangement. Five (22%) of the 24 T-cell tumors expressed βF1, 8 (35%) of the 23 tumors were positive for EBER, and 20 (95%) of the 21 tumors were clonal for T-cell receptor. The overall 1-year survival was 36%. Univariate regression analysis showed that NK-cell lineage, multicentricity, and perforation were associated with poor prognosis. NK-cell lineage (P=0.037) was a poor prognostic factor by multivariate Cox proportional hazard regression analysis. PINKTL in Taiwan is predominantly not enteropathic with a high frequency of perforation, small to medium tumor cell size and cytotoxic phenotype. Primary intestinal NK-cell lymphoma carries a very poor prognosis, and is probably a distinct entity.

Original languageEnglish
Pages (from-to)1230-1240
Number of pages11
JournalAmerican Journal of Surgical Pathology
Issue number8
StatePublished - 08 2009


  • Enteropathy-associated T-cell lymphoma
  • Intestine
  • NK-cell lymphoma
  • Primary intestinal lymphoma
  • T-cell lymphoma
  • Taiwan


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