title: [Type II enteropathy-associated T-cell lymphoma: a clinicopathologic study].
abstract: OBJECTIVE
  To study the clinicopathologic features, immunohistochemical findings, differential diagnosis and prognosis of type II enteropathy-associated T-cell lymphoma (EATL).
  
  
  METHODS
  Fourteen cases of type II EATL encountered in Department of Pathology, Nanjing General Hospital were retrospectively reviewed. The clinical data, histologic features, immunohistochemical findings and follow-up information were analyzed, with literature review.
  
  
  RESULTS
  There were altogether 12 males and 2 females. The median age of patient was 49 years. The sites of involvement included jejunum (10 cases) and ileum/colon (4 cases). The patients often presented with an abdominal mass, abdominal pain, diarrhea and constitutional symptoms such as fever, night sweating and cachexia. There was no clinical evidence of gluten-sensitive enteropathy. Histologically, the lymphoma cells showed full-thickness infiltration of the intestinal wall. They contained round hyperchromatic nuclei and pale cytoplasm. The stroma was minimally inflamed, with or without associated coagulative necrosis. A remarkable finding was the presence of villous atrophy, cryptal hyperplasia and intraepithelial lymphocytosis. Immunohistochemical study showed that the tumor cells expressed CD3, CD43 and CD8 (14/14). Some of them were also positive for CD56 (11/14) and CD30 (2/14). The staining for CD4, CD20, CD79a and myeloperoxidase was negative. A high proliferation index was demonstrated by Ki-67 immunostain. In-situ hybridization for EBER was negative. Follow-up data were available in 9 cases. The duration of follow-up ranged from 6 months to 36 months. Seven patients died within 14 months.
  
  
  CONCLUSIONS
  EATL is a rare type of lymphoma with intestinal involvement. Associated enteropathy is not demonstrated, in contrast to cases encountered in Nordic countries. A correct diagnosis requires evaluation of clinical manifestations, pathologic features and ancillary study results.
authors.affiliation: Department of Pathology, Clinical School of Nanjing University Medical College and Nanjing General Hospital of PLA, Nanjing 210002, China.
authors.forename: Jun
authors.initials: J
authors.lastname: Zhou
authors.forename: Qin
authors.initials: Q
authors.lastname: Shen
authors.forename: Jie
authors.initials: J
authors.lastname: Ma
authors.forename: Xin-hua
authors.initials: XH
authors.lastname: Zhang
authors.forename: Shan-shan
authors.initials: SS
authors.lastname: Shi
authors.forename: Bo
authors.initials: B
authors.lastname: Yu
authors.forename: Xiao-jun
authors.initials: XJ
authors.lastname: Zhou
authors.forename: Qun-li
authors.initials: QL
authors.lastname: Shi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D017252:CD3 Complex / Q000378:metabolism; D016827:CD8 Antigens / Q000378:metabolism; D003937:Diagnosis, Differential; D058527:Enteropathy-Associated T-Cell Lymphoma / Q000235:genetics / Q000276:immunology / Q000473:pathology* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D007078:Ileal Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology* / Q000601:surgery; D007580:Jejunal Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology* / Q000601:surgery; D051917:Leukosialin / Q000378:metabolism; D018442:Lymphoma, B-Cell, Marginal Zone / Q000378:metabolism / Q000473:pathology; D054391:Lymphoma, Extranodal NK-T-Cell / Q000378:metabolism / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D017252:CD3 Complex; D016827:CD8 Antigens; D051917:Leukosialin