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原发性肠道T细胞和自然杀伤细胞淋巴瘤：中国南方79例临床病理及预后特征

Primary intestinal T-cell and natural killer-cell lymphomas: Clinicopathologic and prognostic features of 79 cases in South China.

作者信息

Guo Na, Zhou Chunlu, Wang Yu, Fu Jia, Chen Yueqiong, Wang Fang, Rao Huilan

机构信息

Zhejiang Cancer Hospital Ringgold, Hangzhou, Zhejiang, China.

Chinese Academy of Sciences Ringgold, Beijing, Zhejiang, China.

出版信息

Am J Clin Pathol. 2025 Jan 28;163(1):121-133. doi: 10.1093/ajcp/aqae102.

DOI:10.1093/ajcp/aqae102
PMID:39121027
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11775117/
Abstract

OBJECTIVES

Primary intestinal T-cell and natural killer-cell lymphomas (PITNKLs) are aggressive and make pathologic diagnoses in biopsy specimens challenging. We analyzed different subtypes' clinicopathologic features and treatment outcomes.

METHODS

Seventy-nine PITNKL cases were characterized by clinical, morphologic, and immunohistochemical features.

RESULTS

Among 79 cases of PITNKLs from 2008 to 2017 in our institution, 40 (50.63%) were extranodal NK/T-cell lymphoma, nasal type (ENKTL); 32 (40.51%) monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL); 6 (7.59%) intestinal T-cell lymphoma, not otherwise specified; and 1 (1.27%) indolent T-cell lymphoma of the gastrointestinal tract. Small intestine (n = 47) was the most common site. Monomorphic epitheliotropic intestinal T-cell lymphoma showed distinctive clinicopathologic features from other subtypes with high expression (96.88%) of spleen tyrosine kinase (SYK) and PD-L1 (87.5%) and the poorest prognosis (P < .001). CD30 was highly expressed in ENKTL (9/17, 57.94%) and irrelevant to prognosis (P > .05).

CONCLUSIONS

Cases of PITNKL are biologically heterogeneous; most have a dismal prognosis. SYK and PD-L1 expression might be a significant marker for MEITL and helps differential diagnosis.

摘要

目的

原发性肠道T细胞和自然杀伤细胞淋巴瘤（PITNKLs）具有侵袭性，对活检标本进行病理诊断具有挑战性。我们分析了不同亚型的临床病理特征和治疗结果。

方法

79例PITNKL病例通过临床、形态学和免疫组化特征进行表征。

结果

在我们机构2008年至2017年的79例PITNKL病例中，40例（50.63%）为鼻型结外NK/T细胞淋巴瘤（ENKTL）；32例（40.51%）为单形性亲上皮性肠道T细胞淋巴瘤（MEITL）；6例（7.59%）为未另行特指的肠道T细胞淋巴瘤；1例（1.27%）为胃肠道惰性T细胞淋巴瘤。小肠（n = 47）是最常见的部位。单形性亲上皮性肠道T细胞淋巴瘤表现出与其他亚型不同的临床病理特征，脾酪氨酸激酶（SYK）高表达（96.88%），PD-L1高表达（87.5%），预后最差（P <.001）。CD30在ENKTL中高表达（9/17，57.94%），与预后无关（P >.05）。

结论

PITNKL病例在生物学上具有异质性；大多数预后不佳。SYK和PD-L1表达可能是MEITL的重要标志物，有助于鉴别诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/a065a86d1ae7/aqae102_fig7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/4ff260f31aee/aqae102_fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/0c8a3edd977d/aqae102_fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/a20bf3cc87c7/aqae102_fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/74d22d1fff50/aqae102_fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/71a73d7fc479/aqae102_fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/93f3652b6808/aqae102_fig6a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/a065a86d1ae7/aqae102_fig7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/4ff260f31aee/aqae102_fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/0c8a3edd977d/aqae102_fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/a20bf3cc87c7/aqae102_fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/74d22d1fff50/aqae102_fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/71a73d7fc479/aqae102_fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/93f3652b6808/aqae102_fig6a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f74b/11775117/a065a86d1ae7/aqae102_fig7.jpg

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