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Published on: November 11, 2021
[Clinical analysis of 72 children with Langerhans cell histiocytosis]
Wen-Xuan Jiang1, Fang-Hua Ye1, Yi-Xin Xiao1
1Department of Pediatric Hematology, Xiangya Hospital of Central South University, Changsha 410008, China.
Insights
Pediatric Langerhans cell histiocytosis (LCH) most commonly affects the skull. Risk organ involvement and high platelet counts indicate a poorer prognosis, while the BRAF-V600E mutation shows no relation to outcomes.
Area of Science:
- Pediatric Oncology
- Hematology
- Genetics
Context:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disease.
- Pediatric LCH presents with diverse clinical manifestations and requires understanding of prognostic factors.
Purpose:
- To elucidate the clinical features, treatment effectiveness, and survival outcomes of pediatric Langerhans cell histiocytosis (LCH).
Summary:
- This retrospective study analyzed 72 pediatric LCH cases, identifying skull involvement as most frequent (77.8%). The BRAF-V600E mutation did not correlate with clinical characteristics, efficacy, or prognosis.
- A 5-year overall survival rate of 91.6% and event-free survival (EFS) rate of 67.5% were observed. Risk organ involvement, multisystem disease, and elevated platelet counts (≥450×10^9/L) were linked to poorer prognoses.
- Risk organ involvement emerged as an independent predictor of reduced 5-year EFS.
Impact:
- Findings highlight key prognostic indicators in pediatric LCH, aiding in risk stratification and treatment planning.
- This research underscores the importance of considering organ involvement and platelet count for predicting outcomes in pediatric LCH patients.
Objectives:
To study the clinical characteristics, efficacy, and prognosis of pediatric Langerhans cell histiocytosis (LCH).
Methods:
A retrospective analysis was conducted on 72 children with newly diagnosed LCH.
Results:
The median age of the 72 children was 5 years (range: 0-14 years), with skull involvement being the most common (56 cases, 77.8%). The BRAF-V600E mutation was not associated with clinical characteristics, efficacy, or prognosis (P>0.05). The 5-year overall survival rate was 91.6%±4.2%, and the 5-year event-free survival (EFS) rate was 67.5%±5.8%. The 6-week chemotherapy response rate and 5-year EFS rate were lower in the risk organ involvement group compared to the no risk organ involvement group (P<0.05). The five-year overall survival rates for the group with multi-system involvement and the group with platelet count ≥450×109/L were respectively lower than those for the single-system involvement group and the group with platelet count <450×109/L (P<0.05). Risk organ involvement is an independent risk factor for 5-year EFS (P<0.05).
Conclusions:
Skull is the most commonly affected site in pediatric LCH. The BRAF-V600E mutation is not related to clinical characteristics, efficacy, or prognosis. Elevated platelet count, risk organ involvement, and multisystem involvement are associated with poor prognosis, with risk organ involvement being an independent risk factor for 5-year EFS.
