title: Enteropathy-associated T-cell lymphoma--a clinicopathologic and array comparative genomic hybridization study.
abstract: According to the new World Health Organization classification system, there are 2 types of enteropathy-associated T-cell lymphoma. Type 1 is associated with celiac disease and accounts for the majority of cases in Western countries, whereas type 2 is not associated with celiac disease. To characterize enteropathy-associated T-cell lymphoma types in Korea, we carried out clinicopathologic and immunophenotypic analyses of 8 Koreans with enteropathy-associated T-cell lymphoma and investigated genomic profile using array comparative genomic hybridization. The tumors involved the small intestine in 5 patients and the colorectum in 3 patients. Two patients carried an HLA DQB10302 allele that corresponds to HLA DQ8. None of the patients had gluten-sensitive malabsorption syndrome. Intraepithelial lymphocytosis was observed in all patients. The sizes of the tumor cells were small or small-to-medium in 7 cases and medium-to-large in 1 case. The immunophenotypes of the tumor cells were CD4-CD8+CD56+ in 4 cases, CD4-CD8+CD56- in 1 case, CD4-CD8-CD56+ in 2 cases, and CD4-CD8-CD56- in 1 case. Array comparative genomic hybridization analysis showed that chromosome 9q33-q34.1 gain was present in 4 (80%) of the 5 cases examined. Other recurrent genomic alterations were gain of 6p21.1-21.31 (3/5, 60%), gain of 19q (2/5), and the loss of 3p12.1-p12.2 (2/5) and 3q26.31 (2/5). These results suggest that the most prevalent type of enteropathy-associated T-cell lymphoma in this geographic region is type 2, and the genetic changes associated with it are similar to those in Western countries.
authors.affiliation: Department of Pathology, Samsung Medical Center, Sungkyunkwan University, Kangnamgu, Seoul 135-710, Korea. yhko310@skku.edu
authors.forename: Young Hyeh
authors.initials: YH
authors.lastname: Ko
authors.forename: Sivasundaram
authors.initials: S
authors.lastname: Karnan
authors.forename: Kyeong Mee
authors.initials: KM
authors.lastname: Kim
authors.forename: Cheol Keun
authors.initials: CK
authors.lastname: Park
authors.forename: Eun Suk
authors.initials: ES
authors.lastname: Kang
authors.forename: Young Ho
authors.initials: YH
authors.lastname: Kim
authors.forename: Won Ki
authors.initials: WK
authors.lastname: Kang
authors.forename: Seok Jin
authors.initials: SJ
authors.lastname: Kim
authors.forename: Won Seog
authors.initials: WS
authors.lastname: Kim
authors.forename: Woo Yong
authors.initials: WY
authors.lastname: Lee
authors.forename: Ho Kyung
authors.initials: HK
authors.lastname: Chun
authors.forename: Masao
authors.initials: M
authors.lastname: Seto
mesh_terms: D000328:Adult; D000368:Aged; D000782:Aneuploidy; D024221:Antineoplastic Protocols; D002446:Celiac Disease / Q000235:genetics* / Q000276:immunology / Q000473:pathology*; D055028:Comparative Genomic Hybridization; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D006683:HLA-DQ Antigens / Q000235:genetics; D006684:HLA-DR Antigens / Q000235:genetics; D059811:HLA-DRB1 Chains; D006801:Humans; D016130:Immunophenotyping; D007414:Intestinal Neoplasms / Q000235:genetics* / Q000276:immunology / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000235:genetics* / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012367:RNA, Viral / Q000032:analysis; D056910:Republic of Korea / Q000453:epidemiology; D015996:Survival Rate; D055815:Young Adult
chemical_list: D004273:DNA, Neoplasm; C099537:Epstein-Barr virus encoded RNA 1; D006683:HLA-DQ Antigens; C109756:HLA-DQ8 antigen; D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains; D012367:RNA, Viral