Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation
Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation
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DOI：
10.1182/blood-2009-07-231324
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发表时间：
2010-05-06
期刊：
影响因子：
20.3
通讯作者：
Lennard, Anne L.
中科院分区：
文献类型：
--
作者：
Sieniawski, Michal;Angamuthu, Nithia;Lennard, Anne L.
肠病相关性T细胞淋巴瘤(EATL)是一种罕见的外周T细胞淋巴瘤。目前，EATL尚无标准化的诊断或治疗方案。我们描述了以人群为基础的EATL，并评估了一种积极的化疗和自体干细胞移植(ASCT)的新治疗方法。从1979年起，苏格兰和纽卡斯尔淋巴瘤小组前瞻性地收集了英格兰和苏格兰北部地区所有新诊断的淋巴瘤患者的数据。我们回顾了1994-1998年间所有诊断为EATL的患者的记录，其中54例有EATL的特征。总发病率为每年0.14/10万。治疗方法是全身化疗(主要是基于蒽环类药物的化疗)，其中35名患者接受或不接受手术治疗，19名患者仅接受手术治疗。中位无进展生存期(PFS)3.4个月，总生存期(OS)7.1个月。新的IVE/MTX(异环磷酰胺、长春新碱、依托泊苷/甲氨蝶呤)-ASCT方案从1998年开始试验，用于符合加强治疗条件的患者，纳入26例患者。5年PFS和OS分别为52%和60%，与接受以蒽环类药物为基础的化疗的历史组相比有显著改善(分别为P=0.01和P=0.003)。EATL是一种罕见的淋巴瘤，在常规治疗时预后不良。IVE/MTX-ASCT方案是可行的，毒性可接受，疗效显著改善。(血。2010；115(18)：3664-3670)
Enteropathy associated T-cell lymphoma (EATL) is a rare type of peripheral T-cell lymphoma. At present, there are no standardized diagnostic or treatment protocols for EATL. We describe EATL in a population-based setting and evaluate a new treatment with aggressive chemotherapy and autologous stem cell transplantation (ASCT). From 1979 onward the Scotland and Newcastle Lymphoma Group prospectively collected data on all patients newly diagnosed with lymphoma in the Northern Region of England and Scotland. Between 1994 and 1998, records of all patients diagnosed with EATL were reviewed, and 54 patients had features of EATL. Overall incidence was 0.14/100 000 per year. Treatment was systemic chemotherapy (mostly anthracycline-based chemotherapy) with or without surgery in 35 patients and surgery alone in 19 patients. Median progression-free survival (PFS) was 3.4 months and overall survival (OS) was 7.1 months. The novel regimen IVE/MTX (ifosfamide, vincristine, etoposide/methotrexate)-ASCT was piloted from 1998 for patients eligible for intensive treatment, and 26 patients were included. Five-years PFS and OS were 52% and 60%, respectively, and were significantly improved compared with the historical group treated with anthracycline-based chemotherapy (P = .01 and P = .003, respectively). EATL is a rare lymphoma with an unfavorable prognosis when treated with conventional therapies. The IVE/MTX-ASCT regimen is feasible with acceptable toxicity and significantly improved outcome. (Blood. 2010; 115(18):3664-3670)