淋巴结滤泡辅助T细胞淋巴瘤-血管免疫母细胞型临床病理特征和预后因素分析

Clinicopathological features and prognostic factors of nodal T-follicular helper cell lymphoma，angioimmunoblastic-type

摘要

目的 探讨淋巴结滤泡辅助T细胞淋巴瘤-血管免疫母细胞型（nodal T-follicular helper cell lymphoma，angioimmuno-blastic-type，nTFHL-AI）临床病理特征和预后因素。 方法 回顾性分析63例nTFHL-AI患者临床信息，利用免疫组化（immunohistochemistry，IHC）、原位杂交（in situ hybridization，ISH）和抗原受体基因重排评估nTFHL-AI临床病理特征。使用Cox比例风险回归模型评估预后因素。 结果 免疫染色提示CD4阳性肿瘤细胞例数明显超过CD8（66% vs. 5%）。Epstein-Barr病毒（Epstein-Barr virus，EBV）编码的小RNA（EBV-encoded small RNA，EBER）阳性患者比EBER阴性患者更易表达CXCL13（P=0.006）。5年总体生存期（overall survival，OS）和无进展生存期（progression-free survival，PFS）分别为31%和16%。CXCL13阳性组OS和PFS显著优于CXCL13阴性组（P=0.003、P=0.040）。相反，BCL6阳性表达与较差的OS和PFS相关（P=0.026、P= 0.044），EBER阴性也与较差的OS和PFS相关（P=0.013、P=0.047）。多因素分析表明EBER阴性是OS和PFS独立不良预后因素（P=0.001、P=0.003）。 结论 CXCL13阳性预示nTFHL-AI患者预后良好，而BCL6阳性和EBER阴性与患者预后不良相关，并且EBER阴性是患者预后的独立危险因素。

Abstract

Objective To investigate the clinicopathologic features and prognostic factors of nodal T-follicular helper cell lymphoma，angioimmunoblastic-type（nTFHL-AI）. Methods A retrospective analysis was performed for the clinical information of 63 patients with nTFHL-AI，and immunohistochemistry，in situ hybridization，and antigen receptor gene rearrangement were used to assess the clinicopathological features of nTFHL-AI. The Cox proportional hazards regression model was used to investigate prognostic factors. Results Immunostaining showed that the number of patients with dominant CD4-positive cells was significantly higher than that of patients with dominant CD8-positive cells（66% vs. 5%）. The patients who tested positive for Epstein-Barr virus-encoded small RNA （EBER） showed a higher probability for the expression of CXCL13 compared with their EBER-negative counterparts（P=0.006）. The 5-year overall survival（OS） rate and the progression-free survival（PFS） rate were 31% and 16%，respectively. The CXCL13-positive group had significantly better OS and PFS than the CXCL13-negative group（P=0.003 and 0.040）. On the contrary，positive BCL6 expression was associated with worse OS and PFS（P=0.026 and 0.044），and negative EBER was also associated with worse OS and PFS（P=0.013 and 0.047）. The multivariate analysis showed that negative EBER was an independent negative prognostic factor for both OS and PFS（P=0.001 and 0.003）. Conclusion Positive expression of CXCL13 indicates a favorable prognosis of nTFHL-AI patients，while positive BCL6 and negative EBER are associated with poor prognosis，and negative EBER is an independent risk factor for the prognosis of patients.

关键词

淋巴结滤泡辅助T细胞淋巴瘤-血管免疫母细胞型 / 滤泡辅助T细胞表型 / Epstein-Barr病毒编码的小RNA / 预后

Key words

nodal T-follicular helper cell lymphoma，angioimmunoblastic-type / T-follicular helper cell phenotype / Epstein-Barr virus-encoded small RNA / prognosis

基金资助

重庆医科大学重点资助项目(ZHYX202104)