Type II enteropathy-associated T-cell lymphoma: a multicenter analysis from the Asia Lymphoma Study Group.

Enteropathy-associated T-cell lymphoma (EATL) is a rare primary gastrointestinal T-cell lymphoma. A multicenter study from the Asia Lymphoma Study Group identified 38 EATL patients within a 19-year period. All cases were type II EATL. Men were affected twice as common as women, at a median age of 59 (23-89) years. None had a history of celiac disease. The sites of involvement were small bowel and stomach (5%), small bowel (63%), small and large bowel (16%), and large bowel (18%). Common presenting features were bowel perforation (34%), pain (32%), and obstruction (21%). Lymphomas showed monomorphic neoplastic lymphoid infiltrates that were CD3⁺ (100%), CD56⁺ (91%), TIA-1⁺ (96%), CD4⁻CD8⁺ (63%), CD4⁺CD8⁺ (19%), CD4⁻CD8⁻ (16%), and CD4⁺CD8⁻ (3%). Epstein Barr virus was demonstrable in three cases. Despite chemotherapy and/or surgical resection, the overall response and complete response rates were poor at 46% and 38%. The median overall survival (OS) was 7 months and progression-free-survival (PFS) 1 month. Five patients underwent hematopoietic stem cell transplantation all were alive. Age and the prognostic index for peripheral T-cell lymphoma were not prognostically significant. Good performance status was associated with better OS (P = 0.03), and response to initial treatment led to better OS and PFS (P < 0.001).

Tse E ，Gill H ，Loong F ，Kim SJ ，Ng SB ，Tang T ，Ko YH ，Chng WJ ，Lim ST ，Kim WS ，Kwong YL ... -  《-》

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 ... -  《-》

Enteropathy-associated T-cell lymphoma.

Enteropathy-associated T-cell lymphoma (EATL) is an intestinal tumour of intraepithelial T lymphocytes, usually presenting as a neoplasm composed of large lymphoid cells and often associated with necrosis and an inflammatory background, including large numbers of histiocytes and eosinophils. Intestinal intraepithelial α-β T-cells have been postulated as the normal-cell counterpart for EATL. EATL is the most common neoplastic complication of coeliac disease. The disease is uncommon in most parts of the world, but is seen with greater frequency in those areas with a high prevalence of coeliac disease, in particular Northern Europe. Usually, EATL occurs in adults, and generally present with abdominal pain, often associated with jejunal perforation, weight loss, diarrhoea, or bowel obstruction. EATL is characterized by multifocal presentation in 10-25% of cases. Small-bowel lymphoma is more common than large-bowel or rectal lymphomas. The prognosis of EATL is very poor, with low chemosensitivity, rapid tumour growth and a tendency to dissemination. Moreover, the high incidence of severe postsurgical complications and the poor nutritional and immunological conditions lead to progressive deterioration of these patients, preventing the use of an adequate and effective treatment.

Ferreri AJ ，Zinzani PL ，Govi S ，Pileri SA ... -  《-》

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 ... -  《-》

Retrospective analysis of 23 cases with peripheral T-cell lymphoma, unspecified: clinical characteristics and outcome.

Zaja F ，Russo D ，Silvestri F ，Fanin R ，Damiani D ，Infanti L ，Salmaso F ，Mariuzzi L ，Di Loreto C ，Baccarani M ... -  《HAEMATOLOGICA》