Enteropathy-associated T-cell lymphoma--a clinicopathologic and array comparative genomic hybridization study.
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.
Ko
Young Hyeh
YH
Department of Pathology, Samsung Medical Center, Sungkyunkwan University, Kangnamgu, Seoul 135-710, Korea. yhko310@skku.edu
Karnan
Sivasundaram
S
Kim
Kyeong Mee
KM
Park
Cheol Keun
CK
Kang
Eun Suk
ES
Kim
Young Ho
YH
Kang
Won Ki
WK
Kim
Seok Jin
SJ
Kim
Won Seog
WS
Lee
Woo Yong
WY
Chun
Ho Kyung
HK
Seto
Masao
M
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
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