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).
Department of Medicine, Queen Mary Hospital, Hong Kong, China.
Eric
E
Tse
Harinder
H
Gill
Florence
F
Loong
Seok Jin
SJ
Kim
Siok-Bian
SB
Ng
Tiffany
T
Tang
Young-Hyeh
YH
Ko
Wee-Joo
WJ
Chng
Soon-Thye
ST
Lim
Won Seog
WS
Kim
Yok-Lam
YL
Kwong
D015746:Abdominal Pain / Q000209:etiology; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058527:Enteropathy-Associated T-Cell Lymphoma / Q000276:immunology / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000276:immunology / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D006723:Hong Kong; D006801:Humans; D007415:Intestinal Obstruction / Q000209:etiology; D007416:Intestinal Perforation / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D056910:Republic of Korea; D012189:Retrospective Studies; D017678:Sex Distribution; D012846:Singapore; D016019:Survival Analysis; D055815:Young Adult