title: Primary intestinal T-cell and NK-cell lymphomas: a clinicopathological and molecular study from China focused on type II enteropathy-associated T-cell lymphoma and primary intestinal NK-cell lymphoma.
abstract: In China, which is a non-endemic area for celiac disease, primary intestinal T-cell and NK-cell lymphomas might comprise heterogeneous subtypes. Both type II enteropathy-associated T-cell lymphoma and primary intestinal NK-cell lymphoma are rarely reported and poorly characterized in China. In this study, we examined the clinicopathological and molecular features of 38 cases of primary intestinal T-cell and NK-cell lymphoma in Chinese patients. Based on these findings, we first classified the patients into an NK-cell group (n=6) and a T-cell group (n=32). In the NK-cell group, the mean age was 37 years. All tumors of the NK-cell group were positive for Epstein-Barr virus encoded mRNA in the majority of tumor cells and were polyclonal according to the results of commercial BIOMED-2 T-cell receptor gene rearrangement assays. The survival in the NK-cell group was significantly worse than that of the T-cell group (P=0.0247). Next, 7 tumors of the T-cell group were considered type II enteropathy-associated T-cell lymphoma, while 24 were considered peripheral T-cell lymphoma, not otherwise specified (NOS). In the type II enteropathy-associated T-cell lymphoma group, the mean age was 55 years. Type II enteropathy-associated T-cell lymphoma tumor cells from all seven patients were monomorphic, medium sized. The survival in the type II enteropathy-associated T-cell lymphoma group was significantly worse than that of the peripheral T-cell lymphoma, NOS group (P=0.0181). Multivariate analysis identified NK-cell phenotype, male gender, and CD8 positivity as factors for poor prognosis in our series of primary intestinal T-cell and NK-cell lymphoma patients. In conclusion, most cases of primary intestinal T-cell and NK-cell lymphoma in China are not associated with celiac disease and could be classified to NK-cell group, type II enteropathy-associated T-cell lymphoma group, and peripheral T-cell lymphoma, NOS group. Each group has distinctive histopathological features with prognostic significance.
authors.affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Jian
authors.initials: J
authors.lastname: Sun
authors.forename: Zhaohui
authors.initials: Z
authors.lastname: Lu
authors.forename: Di
authors.initials: D
authors.lastname: Yang
authors.forename: Jie
authors.initials: J
authors.lastname: Chen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002681:China; D058527:Enteropathy-Associated T-Cell Lymphoma / Q000235:genetics / Q000473:pathology* / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D007414:Intestinal Neoplasms / Q000235:genetics / Q000473:pathology* / Q000821:virology; D053208:Kaplan-Meier Estimate; D007694:Killer Cells, Natural / Q000473:pathology*; D016411:Lymphoma, T-Cell, Peripheral / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D011948:Receptors, Antigen, T-Cell / Q000235:genetics; D012307:Risk Factors; D055815:Young Adult
chemical_list: D011948:Receptors, Antigen, T-Cell