Type II enteropathy-associated T-cell lymphoma: A multicenter analysis from the Asia Lymphoma Study Group
Type II enteropathy-associated T-cell lymphoma: A multicenter analysis from the Asia Lymphoma Study Group
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DOI：
10.1002/ajh.23213
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发表时间：
2012-07-01
影响因子：
12.8
通讯作者：
Kwong, Yok-Lam
中科院分区：
文献类型：
--
作者：
Tse, Eric;Gill, Harinder;Kwong, Yok-Lam
肠病相关性T细胞淋巴瘤（EATL）是一种罕见的原发性胃肠道T细胞淋巴瘤。亚洲淋巴瘤研究小组的一项多中心研究在19年内确定了38名EATL患者。所有病例均为II型EATL。男性患病率是女性的两倍，中位年龄为59岁（23-89岁）。无乳糜泻病史。受累部位为小肠和胃（5%）、小肠（63%）、小肠和大肠（16%）和大肠（18%）。常见的表现为肠穿孔（34%）、疼痛（32%）和梗阻（21%）。淋巴瘤显示单形肿瘤性淋巴浸润，为CD 3+（100%）、CD 56+（91%）、TIA-1+（96%）、CD 4CD 8+（63%）、CD 4 + CD 8+（19%）、CD 4CD 8（16%）和CD 4 + CD 8（3%）。三例病例中证实有爱泼斯坦巴尔病毒。尽管化疗和/或手术切除，总体反应和完全反应率为46%和38%。中位总生存期（OS）为7个月，无进展生存期（PFS）为1个月。5例行造血干细胞移植的患者均存活。年龄和外周T细胞淋巴瘤的预后指数无统计学意义。良好的体能状态与较好的OS相关（P = 0.03），对初始治疗的反应导致较好的OS和PFS（P < 0.001）。Am. J. Hematol. 87：663668，2012. (C)2012 Wiley Periodicals，Inc.
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%), CD4CD8+ (63%), CD4+CD8+ (19%), CD4CD8 (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). Am. J. Hematol. 87:663668, 2012. (C) 2012 Wiley Periodicals, Inc.