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

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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.

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单形上皮性肠T细胞淋巴瘤累及中枢神经系统1例并文献复习。

单形上皮性肠T细胞淋巴瘤（MEITL）是一种罕见的消化道原发性T细胞淋巴瘤亚型，其特点是具有高度侵袭性的临床病程。自体造血干细胞移植后的手术、放疗和化疗（CT）是治疗此病的主要方法。然而，目前还没有有效的治疗方法。我们提出一个没有乳糜泻病史的60岁男性患者空肠穿孔的病例。在标本中，观察到表达CD3、CD8和CD56及细胞毒性标志物的中小T细胞弥漫性浸润，影响肠壁各层。确诊为MEITL，患者行6周期CT检查。疾病进展伴另一肠穿孔及中枢神经系统受累。患者在确诊后9个月死亡。通过PubMed检索发现16例相似病例，并描述其临床病理特征。

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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.