Clinical Features and Prognosis of Langerhans Cell Histiocytosis in Children: An Analysis of 31 Cases

Haijin LI; Zhengcheng DUAN; Yan ZHOU; Liufang WANG; Chunhui YANG; Xin TIAN

doi:10.12259/j.issn.2095-610X.S20231215

Volume 44 Issue 12

Dec. 2023

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Haijin LI, Zhengcheng DUAN, Yan ZHOU, Liufang WANG, Chunhui YANG, Xin TIAN. Clinical Features and Prognosis of Langerhans Cell Histiocytosis in Children: An Analysis of 31 Cases[J]. Journal of Kunming Medical University, 2023, 44(12): 95-99. doi: 10.12259/j.issn.2095-610X.S20231215

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Clinical Features and Prognosis of Langerhans Cell Histiocytosis in Children: An Analysis of 31 Cases

doi: 10.12259/j.issn.2095-610X.S20231215

Dept. of Hematology and Oncology，Kunming Children’s Hospital/Children’s Hospital Affiliated to Kunming Medical University，Kunming Yunnan 650228，China

Received Date: 2023-09-19
Available Online: 2023-12-20
Publish Date: 2023-12-25

Abstract

Abstract

Objective To analyze the clinical characteristics of pediatric Langerhans cell histiocytosis（LCH）, and to analyse the expression characteristics and prognosis of BRAF-V600E mutation in LCH. Methods The clinical data of 31 children with LCH admitted to the Department of Hematology, Kunming Children's Hospital from February 2017 to December 2021 were retrospectively analyzed. Reference to LCH Shanghai children’ s medical center - 2011 revision chemotherapy（SCMC - LCH - 2011）, at the same time BRAF mutation - V600E targeted biopsies was treated by Dabrafenib. The clinical features, BRAF-V600E mutation and treatment response between single system LCH（SS-LCH） and multisystem LCH（MS-LCH） were summarized. The Kaplan-Meier method was used to draw the survival curve, and the Log-Rank test was used to compare the survival rate between groups. The Shanghai Children's Medical Center LCH-2011 revised chemotherapy regimen（SCMC-LCH-2011 regimen） was referenced, and the targeted drug Dabrafenib was added to treat those with the BRAF-V600E mutation. The clinical characteristics, BRAF-V600E mutation status, and treatment response between the single-system LCH（SS-LCH） group and the multisystem LCH（MS-LCH） group were summarized. Survival curves were plotted using the Kaplan-Meier method, and the Log-Rank test was used to compare the survival rates between the two groups. Results This study included a total of 31 cases, with 18 males and 13 females. The median age of onset was 10 months（ranging from 1 to 84 months）. 9 cases were SS-LCH, and 22 cases were MS-LCH, with 5 cases experiencing pituitary involvement/diabetes insipidus. Among the 27 cases that underwent BRAF-V600E mutation testing, 20 were positive（3 cases in the SS-LCH group, with a positivity rate of 37.5%; 17 cases in the MS-LCH group, with a positivity rate of 89.5%）. The difference in the BRAF-V600E mutation positivity rate between the two groups was statistically significant（P = 0.011）. The median follow-up time was 24 months（ranging from 3 to 62 months）. The effective rate after 6 weeks of induction chemotherapy was 88.9% in the SS-LCH group（8/9） and 81.8% in the MS-LCH group（18/22）. The observed progression-free rate at the end of the observation period reached 29.0%（9/31）. All three deaths occurred in the MS-LCH group with involvement of high-risk organs. There was no statistically significant difference in the overall survival rate between the SS-LCH and MS-LCH groups, as well as between the BRAF-V600E mutation positive and negative groups（χ² = 1.156, 0.437; P = 0.282, 0.508）. Conclusion LCH in children is more common in infants and young children, with a high incidence of BRAF-V600E gene mutation in affected children, and is often seen in MS-LCH. Dabrafenib may help improve the prognosis of children with BRAF-V600E mutation.
- Langerhans cell histiocytosis,
- Child,
- BRAF-V600E gene

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References(20)

References

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