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The plasma soluble CSF1R level is a promising prognostic indicator for pediatric Langerhans cell histiocytosis
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  • Zhigang Li,
  • Ting Zhu,
  • Chanjuan Wang,
  • Hong-Yun Lian,
  • Hong-Hao Ma,
  • Dong Wang,
  • Tianyou Wang,
  • rui zhang,
  • Lei Cui
Zhigang Li
Beijing Children's Hospital Capital Medical University

Corresponding Author:ericlzg70@hotmail.com

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Ting Zhu
Beijing Children's Hospital Capital Medical University
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Chanjuan Wang
Beijing Children's Hospital Capital Medical University
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Hong-Yun Lian
Beijing Children's Hospital Capital Medical University
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Hong-Hao Ma
Beijing Children's Hospital Capital Medical University
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Dong Wang
Beijing Children's Hospital Capital Medical University
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Tianyou Wang
Beijing Children's Hospital Capital Medical University
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rui zhang
Beijing Children's Hospital Capital Medical University
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Lei Cui
Beijing Children's Hospital Capital Medical University
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Abstract

Langerhans cell histiocytosis (LCH) is a rare hematologic neoplasm characterized by the clonal proliferation of Langerhans-like cells. Colony-stimulating Factor 1 receptor (CSF1R) is a membrane-bound receptor that is highly expressed in LCH cells and tumor-associated macrophages. In this study, a soluble form of CSF1R protein (sCSF1R) was identified by plasma proteome profiling, and its role in evaluating LCH prognosis was explored. We prospectively measured plasma sCSF1R levels in 104 LCH patients and 10 healthy children using ELISA. Plasma sCSF1R levels were greater in LCH patients than in healthy controls ( P < 0.001) and significantly differed among the three disease extents, with the highest level in MS RO+ LCH patients ( P < 0.001). Accordingly, immunofluorescence showed the highest level of membrane-bound CSF1R in MS RO+ patients. Furthermore, the plasma sCSF1R concentration at diagnosis could efficiently predict the prognosis of LCH patients treated with standard first-line treatment (AUC =0.782, P < 0.001). Notably, dynamic monitoring of sCSF1R levels could predict relapse early in patients receiving BRAF inhibitor treatment. In vitro drug sensitivity data showed that sCSF1R increased resistance to Ara-C in THP-1 cells expressing ectopic BRAF-V600E. Overall, the plasma sCSF1R level at diagnosis and during follow-up is of great clinical importance in pediatric LCH patients.
25 Jan 2024Submitted to Pediatric Blood & Cancer
25 Jan 2024Submission Checks Completed
25 Jan 2024Assigned to Editor
25 Jan 2024Review(s) Completed, Editorial Evaluation Pending
25 Jan 2024Reviewer(s) Assigned
03 Feb 2024Editorial Decision: Revise Minor
20 Feb 2024Submission Checks Completed
20 Feb 2024Assigned to Editor
21 Feb 2024Review(s) Completed, Editorial Evaluation Pending