A case report and a literature review about central nervous system involvement in monomorphic epitheliotropic intestinal T cell lymphoma.

IF 0.6 4区医学 Q4 HEMATOLOGY Journal of Hematopathology Pub Date : 2025-02-27 DOI:10.1007/s12308-025-00618-w

Ricard Onieva, Fabiana Aguirre, Carmen Blázquez, Rubén Carrera, Sonia Piernas, Maria Elena Ramila, Alfons Soler, Joan Carles Ferreres, Natalia Papaleo

{"title":"A case report and a literature review about central nervous system involvement in monomorphic epitheliotropic intestinal T cell lymphoma.","authors":"Ricard Onieva, Fabiana Aguirre, Carmen Blázquez, Rubén Carrera, Sonia Piernas, Maria Elena Ramila, Alfons Soler, Joan Carles Ferreres, Natalia Papaleo","doi":"10.1007/s12308-025-00618-w","DOIUrl":null,"url":null,"abstract":"<p><p>Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL) is a rare subtype of primary T cell lymphoma of the digestive tract, characterized by a highly aggressive clinical course. Surgery, radiotherapy, and chemotherapy (CT) following autologous hematopoietic stem cell transplantation are among the treatments selected for this disease. Nevertheless, there is currently no curative therapy. We present a case of a 60-year-old male patient without history of celiac disease who presented a jejunum perforation. In the specimen, a diffuse infiltration of small to medium-sized T cells with CD3, CD8, and CD56 expression and cytotoxic markers was observed, affecting all the layers of intestinal wall. The diagnosis of MEITL was established, and the patient received six cycles of CT. Disease progression with another intestinal perforation and central nervous system involvement was presented. The patient died 9 months after the diagnosis. Sixteen similar cases were found through PubMed search, and we describe their clinicopathological characteristics.</p>","PeriodicalId":51320,"journal":{"name":"Journal of Hematopathology","volume":"18 1","pages":"7"},"PeriodicalIF":0.6000,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Hematopathology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s12308-025-00618-w","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL) is a rare subtype of primary T cell lymphoma of the digestive tract, characterized by a highly aggressive clinical course. Surgery, radiotherapy, and chemotherapy (CT) following autologous hematopoietic stem cell transplantation are among the treatments selected for this disease. Nevertheless, there is currently no curative therapy. We present a case of a 60-year-old male patient without history of celiac disease who presented a jejunum perforation. In the specimen, a diffuse infiltration of small to medium-sized T cells with CD3, CD8, and CD56 expression and cytotoxic markers was observed, affecting all the layers of intestinal wall. The diagnosis of MEITL was established, and the patient received six cycles of CT. Disease progression with another intestinal perforation and central nervous system involvement was presented. The patient died 9 months after the diagnosis. Sixteen similar cases were found through PubMed search, and we describe their clinicopathological characteristics.

查看原文

微信好友朋友圈 QQ好友复制链接

本刊更多论文

求助全文

约1分钟内获得全文去求助

来源期刊

Journal of Hematopathology HEMATOLOGYPATHOLOGY-PATHOLOGY

CiteScore

0.80

自引率

0.00%

发文量

期刊介绍： The Journal of Hematopathology aims at providing pathologists with a special interest in hematopathology with all the information needed to perform modern pathology in evaluating lymphoid tissues and bone marrow. To this end the journal publishes reviews, editorials, comments, original papers, guidelines and protocols, papers on ancillary techniques, and occasional case reports in the fields of the pathology, molecular biology, and clinical features of diseases of the hematopoietic system. The journal is the unique reference point for all pathologists with an interest in hematopathology. Molecular biologists involved in the expanding field of molecular diagnostics and research on lymphomas and leukemia benefit from the journal, too. Furthermore, the journal is of major interest for hematologists dealing with patients suffering from lymphomas, leukemias, and other diseases. The journal is unique in its true international character. Especially in the field of hematopathology it is clear that there are huge geographical variations in incidence of diseases. This is not only locally relevant, but due to globalization, relevant for all those involved in the management of patients.