Lenti Marco Vincenzo, Biagi Federico, Lucioni Marco, Di Sabatino Antonio, Paulli Marco, Corazza Gino Roberto
Department of Internal Medicine, University of Pavia, Fondazione IRCCS Policlinico San Matteo , Pavia , Italy.
Gastroenterology Unit, ICS Maugeri IRCCS, University of Pavia , Pavia , Italy.
Scand J Gastroenterol. 2019 Aug;54(8):965-968. doi: 10.1080/00365521.2019.1647455. Epub 2019 Jul 30.
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an aggressive type of intestinal lymphoma which affects individuals without evidence of enteropathy. In this single-centre case series, we describe the first two cases of MEITL in Caucasian patients suffering from histologically-proven coeliac disease (CD). Original medical records were retrieved and anonymised. All biopsy and surgical MEITL specimens were reviewed by three haematopathologists. Two patients aged 63- and 55-year old at CD diagnosis, subsequently developed a MEITL. MEITL always involved the ileum and was multifocal. Both patients died from complications after surgery, including gastrointestinal bleeding, septic shock and multiorgan failure, with a mean survival since MEITL diagnosis of 15.5 ± 16.3 months. In one case, array-CGH revealed a large deletion on chromosome nine between 9p13.1 and 9p24.1, and a recurrent chromosome gain at 9q33-q34. Our cases indicate that a subset of MEITL may arise in Caucasian patients suffering from CD. The clinical, pathological and molecular features of these cases show a partial overlap with enteropathy-associated T-cell lymphoma.
单形上皮趋化性肠道T细胞淋巴瘤（MEITL）是一种侵袭性肠道淋巴瘤，影响无肠病证据的个体。在这个单中心病例系列中，我们描述了首例两例患有经组织学证实的乳糜泻（CD）的白种人患者发生MEITL的情况。检索并匿名化原始病历。所有MEITL活检和手术标本均由三名血液病理学家进行复查。两名患者在CD诊断时分别为63岁和55岁，随后发生了MEITL。MEITL总是累及回肠且为多灶性。两名患者均死于手术后并发症，包括胃肠道出血、感染性休克和多器官功能衰竭，自MEITL诊断后的平均生存期为15.5±16.3个月。在一例中，阵列比较基因组杂交（array-CGH）显示9号染色体9p13.1和9p24.1之间有大片段缺失，9q33-q34有反复的染色体增加。我们的病例表明，一部分MEITL可能发生在患有CD的白种人患者中。这些病例的临床、病理和分子特征与肠病相关T细胞淋巴瘤有部分重叠。
