Detailed clinicopathological characteristics and possible lymphomagenesis of type II intestinal enteropathy-associated T-cell lymphoma in Japan.

Twenty-six Japanese cases of type II enteropathy-associated T-cell lymphoma (EATL) were examined. Multiple tumors throughout the small intestine were found in 15 patients (58%) and duodenal and colonic mucosal lesions in 8 and 6 cases, respectively. Histologically, intramucosal tumor spread and a zone of neoplastic intraepithelial lymphocytes (IELs) neighboring the main transmural tumors were detected in 20 (91%) and 17 (77%) of the 22 cases examined, respectively. Inside and outside the IEL zone, some degree of enteropathy with many reactive small IELs and villous atrophy was detected in 11 cases (50%). Immunohistologically, many CD56/CD8-positive small IELs were found in the enteropathic lesions of 4 (36%) and 7 (64%) of these 11 cases. Lymphoma cells expressed tyrosine kinase receptor c-Met, serial phosphorylated (p)-mitogen-activated protein kinase/extracellular signal-regulated kinase, c-Myc, and Bcl2 in 18 (78%), 21 (91%), 11 (42%), and 19 (73%) of the total cases, respectively. By fluorescence in situ hybridization, chromosomal loci 7q31 (c-Met) and 8q24 (c-Myc) were amplified in 11 (65%) and 12 (71%) of the 17 cases analyzed. Gain of 7q31 and c-Met expression were significantly (P < .01) higher than in peripheral CD8-positive T-cell or CD56-positive natural killer-cell lymphomas. Enteropathy was seen near the IEL zone in type II EATL, and activation of the c-Met, mitogen-activated protein kinase/extracellular signal-regulated kinase-mitogen-activated protein kinase pathway, and c-Myc-Bcl2-mediated cell survival may play important roles in lymphomagenesis, converting enteropathy to type II EATL. Seven cases in the early clinical stages I and II-1 showed significantly (P < .01) better prognoses than did those in the advanced stages. Early detection of the mucosal lesions and tumors may improve patient prognosis.

Kikuma K ，Yamada K ，Nakamura S ，Ogami A ，Nimura S ，Hirahashi M ，Yonemasu H ，Urabe S ，Naito S ，Matsuki Y ，Sadahira Y ，Takeshita M ... -  《-》

Type II enteropathy-associated T-cell lymphoma: a distinct aggressive lymphoma with frequent γδ T-cell receptor expression.

Chan JK ，Chan AC ，Cheuk W ，Wan SK ，Lee WK ，Lui YH ，Chan WK ... -  《-》

Type II EATL (epitheliotropic intestinal T-cell lymphoma): a neoplasm of intra-epithelial T-cells with predominant CD8αα phenotype.

Tan SY ，Chuang SS ，Tang T ，Tan L ，Ko YH ，Chuah KL ，Ng SB ，Chng WJ ，Gatter K ，Loong F ，Liu YH ，Hosking P ，Cheah PL ，Teh BT ，Tay K ，Koh M ，Lim ST ... -  《-》

Multiple lesions of gastrointestinal tract invasion by monomorphic epitheliotropic intestinal T-cell lymphoma, accompanied by duodenal and intestinal enteropathy-like lesions and microscopic lymphocytic proctocolitis: a case series.

Ishibashi H ，Nimura S ，Kayashima Y ，Takamatsu Y ，Aoyagi K ，Harada N ，Kadowaki M ，Kamio T ，Sakisaka S ，Takeshita M ... -  《-》

Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan.

Tomita S ，Kikuti YY ，Carreras J ，Kojima M ，Ando K ，Takasaki H ，Sakai R ，Takata K ，Yoshino T ，Bea S ，Campo E ，Nakamura N ... -  《-》