EXPRESSION AND CLINICAL CORRELATION OF PD-1/PD-L1 AND VE1(BRAFP.V600E) IN PEDIATRIC LANGERHANS CELL HISTIOCYTOSIS
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 correlat...
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| Format: | Article |
| Language: | English |
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PAGEPress Publications
2023-04-01
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| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
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| Online Access: | http://www.mjhid.org/index.php/mjhid/article/view/5164 |
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| author | sneha tandon Sheila Weitzman Brooklyn Joyce Bryan Maguire Derek Stephens James Whitlock Cynthia Hawkins Bo-Yee Ngan Oussama Abla |
| author_facet | sneha tandon Sheila Weitzman Brooklyn Joyce Bryan Maguire Derek Stephens James Whitlock Cynthia Hawkins Bo-Yee Ngan Oussama Abla |
| author_sort | sneha tandon |
| collection | DOAJ |
| description |
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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| format | Article |
| id | doaj-art-9f18f64f6b2044e8bab771ac0d2a1bbf |
| institution | Kabale University |
| issn | 2035-3006 |
| language | English |
| publishDate | 2023-04-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Mediterranean Journal of Hematology and Infectious Diseases |
| spelling | doaj-art-9f18f64f6b2044e8bab771ac0d2a1bbf2025-01-03T00:30:36ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062023-04-0115110.4084/MJHID.2023.035EXPRESSION AND CLINICAL CORRELATION OF PD-1/PD-L1 AND VE1(BRAFP.V600E) IN PEDIATRIC LANGERHANS CELL HISTIOCYTOSIS sneha tandon0Sheila Weitzman 1Brooklyn JoyceBryan MaguireDerek StephensJames WhitlockCynthia Hawkins Bo-Yee NganOussama Abla 2a:1:{s:5:"en_US";s:23:"Barts Health NHS Trust ";}The Hospital for Sick ChildrenThe Hospital for Sick Children 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. http://www.mjhid.org/index.php/mjhid/article/view/5164Langerhans cell histiocytosisPaediatricPD-1PD-L1VE1(BRAFp.V600E)Histiocytosis |
| spellingShingle | sneha tandon Sheila Weitzman Brooklyn Joyce Bryan Maguire Derek Stephens James Whitlock Cynthia Hawkins Bo-Yee Ngan Oussama Abla EXPRESSION AND CLINICAL CORRELATION OF PD-1/PD-L1 AND VE1(BRAFP.V600E) IN PEDIATRIC LANGERHANS CELL HISTIOCYTOSIS Mediterranean Journal of Hematology and Infectious Diseases Langerhans cell histiocytosis Paediatric PD-1 PD-L1 VE1(BRAFp.V600E) Histiocytosis |
| title | EXPRESSION AND CLINICAL CORRELATION OF PD-1/PD-L1 AND VE1(BRAFP.V600E) IN PEDIATRIC LANGERHANS CELL HISTIOCYTOSIS |
| title_full | EXPRESSION AND CLINICAL CORRELATION OF PD-1/PD-L1 AND VE1(BRAFP.V600E) IN PEDIATRIC LANGERHANS CELL HISTIOCYTOSIS |
| title_fullStr | EXPRESSION AND CLINICAL CORRELATION OF PD-1/PD-L1 AND VE1(BRAFP.V600E) IN PEDIATRIC LANGERHANS CELL HISTIOCYTOSIS |
| title_full_unstemmed | EXPRESSION AND CLINICAL CORRELATION OF PD-1/PD-L1 AND VE1(BRAFP.V600E) IN PEDIATRIC LANGERHANS CELL HISTIOCYTOSIS |
| title_short | EXPRESSION AND CLINICAL CORRELATION OF PD-1/PD-L1 AND VE1(BRAFP.V600E) IN PEDIATRIC LANGERHANS CELL HISTIOCYTOSIS |
| title_sort | expression and clinical correlation of pd 1 pd l1 and ve1 brafp v600e in pediatric langerhans cell histiocytosis |
| topic | Langerhans cell histiocytosis Paediatric PD-1 PD-L1 VE1(BRAFp.V600E) Histiocytosis |
| url | http://www.mjhid.org/index.php/mjhid/article/view/5164 |
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