Loghavi Sanam, Medeiros L Jeffrey
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Hum Pathol. 2025 Feb;156:105720. doi: 10.1016/j.humpath.2025.105720. Epub 2025 Jan 24.
We provide an overview of recent advances in the diagnosis and classification of intestinal T- and NK-cell lymphomas and lymphoproliferative disorders that primarily involve the gastrointestinal (GI) tract. It should be mentioned that systemic T- and NK-cell lymphomas can present initially in the GI tract but are not the focus of this review. Here, we focus on the clinical and pathologic findings of enteropathy-associated T-cell lymphoma, monomorphic epitheliotropic intestinal T-cell lymphoma, intestinal T-cell lymphoma, not otherwise specified, indolent T-cell lymphoma of the GI tract and indolent NK-cell lymphoproliferative disorder of the GI tract (previously known as NK-cell enteropathy). These diseases are uncommon, and their clinical manifestations and pathologic features can show overlap, but are associated with different outcomes making accurate diagnosis essential. In most cases, the diagnosis of intestinal T- and NK-cell lymphomas and lymphoproliferative disorders requires integrating the clinical context with the morphologic, immunophenotypic, and molecular alterations. In this review, we also emphasize the current understanding of the genetic drivers of these diseases.
我们概述了肠道T细胞和NK细胞淋巴瘤以及主要累及胃肠道（GI）的淋巴增殖性疾病在诊断和分类方面的最新进展。应当提及的是，系统性T细胞和NK细胞淋巴瘤最初可出现在胃肠道，但并非本综述的重点。在此，我们重点关注肠病相关T细胞淋巴瘤、单形性嗜上皮性肠道T细胞淋巴瘤、未另行指定的肠道T细胞淋巴瘤、胃肠道惰性T细胞淋巴瘤以及胃肠道惰性NK细胞淋巴增殖性疾病（以前称为NK细胞肠病）的临床和病理表现。这些疾病并不常见，其临床表现和病理特征可能会有重叠，但与不同的预后相关，因此准确诊断至关重要。在大多数情况下，肠道T细胞和NK细胞淋巴瘤以及淋巴增殖性疾病的诊断需要将临床背景与形态学、免疫表型和分子改变相结合。在本综述中，我们还强调了对这些疾病遗传驱动因素的当前认识。
