Loghavi Sanam, Medeiros L Jeffrey
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States.
Am J Clin Pathol. 2025 Jul 11;164(1):36-45. doi: 10.1093/ajcp/aqaf016.
To review and discuss cases submitted to the 2023 Society of Hematopathology/European Association for Haematopathology workshop session entitled "Non-cutaneous Cytotoxic T-cell Lymphomas Including Hepatosplenic T-cell Lymphoma and Intestinal T-cell Lymphomas.
A total of 45 cases were submitted by various contributors. These cases included clinicopathologic, immunophenotypic and molecular data.
Cases submitted included 12 hepatosplenic T-cell lymphoma (HSTCL) and 22 intestinal T or NK cell lymphomas or lymphoproliferative disorders (LPD) of various types. The latter group included 12 monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), 4 indolent NK-cell LPD of the gastrointestinal (GI) tract, 3 indolent (clonal) T-cell lymphoma of the GI tract, 1 refractory celiac disease type 2, 1 enteropathy-associated T-cell lymphoma and 1 intestinal T-cell lymphoma not otherwise specified. There were also 11 miscellaneous cases that did not readily fit into well known diagnostic categories but raised questions about diagnostic criteria or biology or which elucidated aspects of differential diagnosis.
The cases submitted were instructive and helped to further characterize and, in some cases, expand these entities. We suggest that HSTCL is a disease with well recognized clinicopathologic features and genetic features. Patients were older than is reflected in the literature. The cases of MEITL in this workshop came mostly from western and/or in industrialized nations. SETD2 mutation or loss of H3K36me3 by immunohistochemistry as a surrogate for this mutation is very common and a helpful diagnostic tool in MEITL. A surprising finding was that some patients with NK-cell LPD of the GI tract exhibited aggressive clinical features including 1 patient who had disease dissemination to the lungs and bile duct.
回顾并讨论提交至2023年血液病理学学会/欧洲血液病理学协会研讨会中题为“非皮肤细胞毒性T细胞淋巴瘤，包括肝脾T细胞淋巴瘤和肠道T细胞淋巴瘤”的病例。
不同贡献者共提交了45例病例。这些病例包含临床病理、免疫表型和分子数据。
提交的病例包括12例肝脾T细胞淋巴瘤（HSTCL）以及22例各种类型的肠道T或NK细胞淋巴瘤或淋巴增殖性疾病（LPD）。后一组包括12例单形性亲上皮性肠道T细胞淋巴瘤（MEITL）、4例胃肠道惰性NK细胞LPD、3例胃肠道惰性（克隆性）T细胞淋巴瘤、1例2型难治性乳糜泻、1例肠病相关T细胞淋巴瘤和1例未另行特指的肠道T细胞淋巴瘤。还有11例杂项病例，它们不易归入已知的诊断类别，但引发了关于诊断标准或生物学的问题，或阐明了鉴别诊断的各个方面。
提交的病例具有指导意义，有助于进一步明确这些实体的特征，在某些情况下还能对其进行扩展。我们认为HSTCL是一种具有公认临床病理特征和遗传特征的疾病。患者年龄比文献中所反映的要大。本次研讨会中的MEITL病例大多来自西方和/或工业化国家。作为该突变替代指标的SETD2突变或免疫组化检测H3K36me3缺失在MEITL中非常常见，是一种有用的诊断工具。一个令人惊讶的发现是，一些胃肠道NK细胞LPD患者表现出侵袭性临床特征，包括1例疾病扩散至肺部和胆管的患者。
