Min Gi-June, Oh Ye Eun, Jeon Youngwoo, Kim Tong Yoon, Kim Byung-Su, Kwag Daehun, Park Sung-Soo, Park Silvia, Yoon Jae-Ho, Lee Sung-Eun, Cho Byung-Sik, Eom Ki-Seong, Kim Yoo-Jin, Lee Seok, Kim Hee-Je, Min Chang-Ki, Lee Jong Wook, Cho Seok-Goo
Department of Hematology, Seoul St. Mary's Hematology Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Department of Hematology, Yeouido St. Mary's Hematology Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Front Oncol. 2024 Nov 21;14:1388623. doi: 10.3389/fonc.2024.1388623. eCollection 2024.
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare, aggressive subtype of primary gastrointestinal T-cell lymphoma. Owing to the absence of symptoms characteristic of MEITL, diagnosis can be challenging, and the low response rate to conventional chemotherapy leads to an abysmal prognosis. This study aimed to define the clinicopathologic characteristics of MEITL in Korea, evaluate the clinical outcomes of intensive chemotherapy with and without hematopoietic stem cell transplantation (HSCT), and explore prognostic factors.
This single-center retrospective study examined the clinical data of 35 patients diagnosed with MEITL at Seoul St. Mary's Hospital from May 2012 to May 2023.
We included 22 men and 13 women (median age: 59 years; range: 37-79 years). Many patients exhibited acute abdominal pain (n=23, 65.7%) related to bowel perforation (n=21, 60.0%). Most patients (30/35, 85.7%) underwent surgical intervention to diagnose MEITL, whereas only five were diagnosed via endoscopic evaluation. Of the 32 patients receiving first-line therapy, 4 died before assessment, 10 achieved a complete response (CR), 6 had a relapse, and 18 exhibited progressive disease (PD). Seven of 10 patients received upfront HSCT, either autologous (auto-HSCT, n=4) or allogeneic (allo-HSCT, n=3). All four patients on auto-HSCT died after relapse. All three patients who received allo-HSCT maintained a CR by the final follow-up. Three of 6 patients who relapsed and 13 of 18 exhibiting PD received salvage therapy; one patient on salvage auto-HSCT with cytokine-induced killer cell infusion has survived progression free. Salvage allo-HSCT was performed on 6 of 16 patients; among them, 2 achieved a CR, 2 died after relapse, and 2 died owing to septic shock while maintaining a CR. The remaining patients, who received salvage therapy without HSCT, mostly died owing to PD. The median overall survival was 12.1 months, and the median follow-up was 33.2 months. The 1- and 5-year overall survival was 50.9% and 13.3%, respectively.
MEITL is an aggressive disease resistant to conventional therapy. Therefore, intensive chemotherapy followed by upfront allo-HSCT should be considered upon diagnosis. These findings underscore the need for novel therapeutic strategies and further investigation into optimizing treatment protocols for MEITL.
单形性上皮趋化性肠道T细胞淋巴瘤（MEITL）是原发性胃肠道T细胞淋巴瘤中一种罕见的侵袭性亚型。由于缺乏MEITL的特征性症状，诊断具有挑战性，且对传统化疗的低反应率导致预后极差。本研究旨在明确韩国MEITL的临床病理特征，评估有或无造血干细胞移植（HSCT）的强化化疗的临床结局，并探索预后因素。
这项单中心回顾性研究检查了2012年5月至2023年5月在首尔圣母医院诊断为MEITL的35例患者的临床资料。
我们纳入了22名男性和13名女性（中位年龄：59岁；范围：37 - 79岁）。许多患者表现出与肠穿孔相关的急性腹痛（n = 23，65.7%）（n = 21，60.0%）。大多数患者（30/35，85.7%）接受了手术干预以诊断MEITL，而只有5例通过内镜评估确诊。在接受一线治疗的32例患者中，4例在评估前死亡，10例达到完全缓解（CR），6例复发，18例表现为疾病进展（PD）。10例患者中有7例接受了前期HSCT，其中自体造血干细胞移植（auto - HSCT，n = 4）或异基因造血干细胞移植（allo - HSCT，n = 3）。4例接受auto - HSCT的患者均在复发后死亡。所有3例接受allo - HSCT的患者在最后随访时维持CR。6例复发患者中的3例和18例表现为PD的患者中的13例接受了挽救治疗；1例接受挽救性auto - HSCT并输注细胞因子诱导的杀伤细胞的患者无进展生存。16例患者中有6例接受了挽救性allo - HSCT；其中，2例达到CR，2例在复发后死亡，2例在维持CR时因感染性休克死亡。其余接受无HSCT挽救治疗的患者大多因PD死亡。中位总生存期为12.1个月，中位随访时间为33.2个月。1年和5年总生存率分别为50.9%和13.3%。
MEITL是一种对传统治疗耐药的侵袭性疾病。因此，诊断时应考虑强化化疗后进行前期allo - HSCT。这些发现强调了对MEITL需要新的治疗策略并进一步研究优化治疗方案。
