Division of Hematology-Oncology, Department of Medicine, Chung-Ang University, Seoul, South Korea.
Division of Hematology and Oncology, Department of Internal Medicine, Institute of Health Science, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, South Korea.
Ann Hematol. 2019 Nov;98(11):2541-2550. doi: 10.1007/s00277-019-03791-y. Epub 2019 Sep 6.
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a provisional entity in the 2017 World Health Organization classifications. To further elucidate the clinicopathologic features of this new disease, we carried out a retrospective, multicenter analysis of 42 patients with MEITL. The median age of the patients was 59 years (range, 20-84 years), and 27 patients (64 %) were male. Thirty-two patients (76 %) were Ann-Arbor stages I-II and 28 (67 %) were Lugano stages I-II. The most frequent site of involvement was the jejunum (N = 21). Most cases expressed CD8 (79 %) and CD56 (95 %) and did not express CD30 (5 %) or EBER (0 %). The median progression-free survival was 6.9 months (95 % CI 4.3-9.6); the median OS was 14.8 months (2.4-27.2). Thirty-two patients (76 %) underwent surgery and 37 (88 %) received chemotherapy. A complete response (CR) rate was 38 %. Sixteen patients had undergone autologous stem cell transplantation (ASCT). Relapse or progression was documented in 24 cases, most frequently in the primary site (N = 23). Four cases showed central nervous system relapse. Age over 55 years, poor performance scale, advanced Lugano stage (II-IV), not achieving CR, and not receiving ASCT were associated with inferior OS. While the optimal management of MEITL remains undetermined, achieving CR and consolidative ASCT seem essential. As CHOP might be insufficient for achieving CR, more efficient combinations should be investigated. Additionally, considering the frequent local failure and CNS relapse, novel therapeutic approaches are required to improve survival.
单一表型上皮样肠道 T 细胞淋巴瘤（MEITL）是 2017 年世界卫生组织分类中的一个暂定实体。为了进一步阐明这种新疾病的临床病理特征，我们对 42 例 MEITL 患者进行了回顾性、多中心分析。患者的中位年龄为 59 岁（范围，20-84 岁），27 例（64%）为男性。32 例（76%）为 Ann-Arbor 分期 I-II 期，28 例（67%）为 Lugano 分期 I-II 期。最常见的受累部位是空肠（N=21）。大多数病例表达 CD8（79%）和 CD56（95%），不表达 CD30（5%）或 EBER（0%）。无进展生存期的中位数为 6.9 个月（95%CI 4.3-9.6）；总生存期的中位数为 14.8 个月（2.4-27.2）。32 例（76%）患者接受了手术，37 例（88%）患者接受了化疗。完全缓解（CR）率为 38%。16 例患者接受了自体干细胞移植（ASCT）。24 例患者记录到复发或进展，最常见于原发部位（N=23）。4 例患者出现中枢神经系统复发。年龄>55 岁、表现状态差、高级 Lugano 分期（II-IV 期）、未达到 CR 和未接受 ASCT 与较差的 OS 相关。虽然 MEITL 的最佳治疗方法尚未确定，但达到 CR 和巩固性 ASCT 似乎是必要的。由于 CHOP 可能不足以达到 CR，应研究更有效的联合治疗方案。此外，考虑到频繁的局部失败和中枢神经系统复发，需要新的治疗方法来提高生存率。
