Fetal and Pediatric Pathology ( IF 1.1 ) Pub Date : 2020-05-13 , DOI: 10.1080/15513815.2020.1764680 Gülen Gül 1, 2 , Dilek Ince 1 , Nur Olgun 1 , Erdener Ozer 3
Abstract
Background: Reed-Sternberg cells can escape from the immune system by enhancement of the expression of PD-L1 and PD-L2. Objectives: The aim of the present study was to investigate the significance PD-L1 and PD-L2 gene mutations in childhood Hodgkin Lymphoma’s (HL). Methods: The study included 39 pediatric classical HL cases. PD-L1 and PD-L2 mutations were determined by Sanger sequencing. Clinicopathological parameters were obtained from patients’ records. Results: Eight cases (20.5%) showed p.R260C mutations, and three (7.7%) p.R234L in the exome 5 of PD-L1 gene. None of the cases had PD-L2 mutations. p.R260C mutation exhibited a significant relationship with older age and nodular sclerosing (NS) histology and was associated with longer event free survival. Conclusions: Although PD-L1 mutational status did not show statistically significance with well-established prognostic factors, our preliminary data indicate that p.R260C mutation of PD-L1 gene may be associated with longer event free survival in older patients and NS histology in pediatric HL.
中文翻译:
小儿霍奇金淋巴瘤中的 PD-L1 和 PD-L2 突变:它们有任何预后意义吗?
摘要
背景: Reed-Sternberg 细胞可以通过增强 PD-L1 和 PD-L2 的表达来逃离免疫系统。目的:本研究的目的是探讨PD-L1和PD-L2基因突变在儿童霍奇金淋巴瘤 (HL) 中的意义。方法:该研究包括 39 例儿科经典 HL 病例。通过 Sanger 测序确定PD-L1和PD-L2突变。从患者的记录中获得临床病理学参数。结果: 8例(20.5%)p.R260C突变,3例(7.7%)p.R234L在PD-L1基因外显子组5。所有病例均未出现PD-L2突变。p.R260C 突变与老年和结节硬化 (NS) 组织学表现出显着关系,并与更长的无事件生存期相关。结论:虽然PD-L1突变状态在已确定的预后因素中没有显示出统计学意义,但我们的初步数据表明PD-L1基因的 p.R260C 突变可能与老年患者的较长无事件生存期和儿科的 NS 组织学相关HL。