PD-1/PD-L1和VE1（BRAFp.V600E）在儿童朗格汉斯细胞组织细胞增多症中的表达及临床相关性
Expression and Clinical Correlation of PD-1/PD-L1 and VE1(BRAFp.V600E) in Pediatric Langerhans Cell Histiocytosis.
作者信息
Tandon Sneha, Weitzman Sheila, Joyce Brooklyn, Mcguire Bryan, Stephens Derek, Whitlock James, Hawkins Cynthia, Ngan Bo Yee, Abla Oussama
机构信息
Division of Paediatric Hematology/Oncology, The Royal London Hospital, Barts Health NHS Trust, London, United Kingdom.
Division of Hematology/Oncology, The Hospital for Sick Children, University of Toronto, Canada.
出版信息
Mediterr J Hematol Infect Dis. 2023 May 1;15(1):e2023035. doi: 10.4084/MJHID.2023.035. eCollection 2023.
BACKGROUND AND OBJECTIVES
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm with a wide spectrum of clinical presentations. Programmed Cell Death-1 (PD-1) receptor and its ligand (PD-L1) are overexpressed in LCH, but their clinical significance is unknown. We performed a clinical correlation study of PD-1/PD-L1 and VE1(BRAFp.V600E) expression in 131 children with LCH.
METHODS
A total of 111 samples were tested for PD-1/PD-L1 and 109 for VE1(BRAFp.V600E) mutant protein by immunohistochemistry.
RESULTS
PD-1, PD-L1 and VE1(BRAFp.V600E) positivity was observed in 40.5%, 31.53% and 55%, respectively. PD-1/ PD-L1 expression showed no significant effect on the rate of disease reactivations, early response to therapy or late sequelae. The 5-year EFS was not statistically different between patients with PD-1 positive compared to those with PD-1 negative tumours (47.7% vs.58.8%, p=0.17). Similar 5-year EFS rates were also seen in those who were PD-L1 positive compared to PD-L1 negative cases (50.5% vs.55.5%, p=0.61). VE1(BRAFp.V600E) positivity was associated with a significantly higher frequency of risk-organ involvement (p=0.0053), but no significant effect on early response to therapy or rates of reactivations or late sequelae.
CONCLUSIONS
Our study showed no significant correlation between VE1(BRAFp.V600E) expression, PD-1 and PD-L1 and clinical outcome in pediatric LCH.
背景与目的
朗格汉斯细胞组织细胞增多症（LCH）是一种具有广泛临床表现的炎症性髓系肿瘤。程序性细胞死亡蛋白1（PD-1）受体及其配体（PD-L1）在LCH中过表达，但其临床意义尚不清楚。我们对131例LCH患儿的PD-1/PD-L1和VE1（BRAF p.V600E）表达进行了临床相关性研究。
方法
通过免疫组织化学对111份样本检测PD-1/PD-L1，对109份样本检测VE1（BRAF p.V600E）突变蛋白。
结果
PD-1、PD-L1和VE1（BRAF p.V600E）阳性率分别为40.5%、31.53%和55%。PD-1/PD-L1表达对疾病复发率、治疗早期反应或晚期后遗症无显著影响。PD-1阳性肿瘤患者与PD-1阴性肿瘤患者的5年无事件生存率无统计学差异（47.7%对58.8%，p=0.17）。PD-L1阳性患者与PD-L1阴性患者的5年无事件生存率也相似（50.5%对55.5%，p=0.61）。VE1（BRAF p.V600E）阳性与风险器官受累频率显著更高相关（p=0.0053），但对治疗早期反应、复发率或晚期后遗症无显著影响。
结论
我们的研究表明，在儿童LCH中，VE1（BRAF p.V600E）表达、PD-1和PD-L1与临床结局之间无显著相关性。
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