Original Articles
Vol. 15 No. 1 (2023): Review Articles, Original Article, Scientific Letter, Case Reports Letter to the Editor
EXPRESSION AND CLINICAL CORRELATION OF PD-1/PD-L1 AND VE1(BRAFP.V600E) IN PEDIATRIC LANGERHANS CELL HISTIOCYTOSIS
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Received: September 27, 2022
Accepted: April 24, 2023
Accepted: April 24, 2023
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Background: 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 a clinical outcome in pediatric LCH.
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How to Cite
“EXPRESSION AND CLINICAL CORRELATION OF PD-1/PD-L1 AND VE1(BRAFP.V600E) IN PEDIATRIC LANGERHANS CELL HISTIOCYTOSIS ” (2023) Mediterranean Journal of Hematology and Infectious Diseases, 15(1), p. e2023035. doi:10.4084/MJHID.2023.035.
Copyright (c) 2023 sneha tandon, Sheila Weitzman , Brooklyn Joyce, Bryan Maguire, Derek Stephens, James Whitlock, Cynthia Hawkins , Bo-Yee Ngan, Oussama Abla
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