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原发性肠道 T 细胞和 NK 细胞淋巴瘤:中国南方 79 例的临床病理和预后特征

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

PubMed 2025/01/28(内容时间) Am J Clin Pathol Q2 · IF 2.3(JCR 2025)

研究概要

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

研究思路结论见上方概要

原发性肠道T细胞和NK 细胞淋巴瘤(PITNKLs)具有侵袭性,在活检标本中做出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局。

79例PITNKL病例通过临床、形态学和免疫组化特征进行了表征。

在我们机构2008年至2017年的79例PITNKLs中,40例(50.63%)为结外NK/T细胞淋巴瘤,鼻型(ENKTL);32例(40.51%)为单形性嗜上皮性肠道T细胞淋巴瘤(MEITL);6例(7.59%)为肠道T细胞淋巴瘤,非特指型;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。单形性嗜上皮性肠道T细胞淋巴瘤与其他亚型相比表现出独特的临床病理特征,脾酪氨酸激酶(SYK)高表达(96.88%)和PD-L1高表达(87.5%),且预后最差(P < .001)。CD30在ENKTL中高表达(9/17,57.94%),与预后无关(P > .05)。

展开英文摘要原文

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.

论文信息

作者
Guo N、Zhou C、Wang Y、Fu J、Chen Y、Wang F、Rao H
第一作者单位
Zhejiang Cancer Hospital Ringgold, Hangzhou, Zhejiang, China.China
通讯作者单位
Department of Pathology, Sun Yat-sen University Cancer Center, Guangzhou, Guangdong, China.China
期刊
American journal of clinical pathology2025 Jan 28
原文标识
PubMed 39121027 · DOI 10.1093/ajcp/aqae102