title: NK-cell lineage predicts poor survival in primary intestinal NK-cell and T-cell lymphomas.
abstract: 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 betaF1, 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 betaF1, 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.
authors: Department of Pathology, Chi-Mei Medical Center, Yung-Kang City, Tainan County, Taiwan 710. cmh5301@mail.chimei.org.tw
authors: Shih-Sung
authors: SS
authors: Chuang
authors: Sheng-Tsung
authors: ST
authors: Chang
authors: Wen-Yu
authors: WY
authors: Chuang
authors: Wan-Ting
authors: WT
authors: Huang
authors: Pin-Pen
authors: PP
authors: Hsieh
authors: Mei-Hua
authors: MH
authors: Tsou
authors: Yung-Liang
authors: YL
authors: Liao
authors: Shu-Hui
authors: SH
authors: Lin
authors: Yen-Chuan
authors: YC
authors: Hsieh
authors: Chin-Li
authors: CL
authors: Lu
authors: Ming-Jen
authors: MJ
authors: Sheu
authors: Hongxiang
authors: H
authors: Liu
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D003131:Combined Modality Therapy; D013505:Digestive System Surgical Procedures; D005260:Female; D006566:Herpesviridae Infections / Q000150:complications; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D007414:Intestinal Neoplasms / Q000401:mortality* / Q000473:pathology* / Q000821:virology; D053208:Kaplan-Meier Estimate; D007694:Killer Cells, Natural / Q000378:metabolism / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000401:mortality* / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis
chemical_list: D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor