Translational pediatrics · 2026
Clinical and prognostic significance of initial fever in children with Langerhans cell histiocytosis.
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Li Na, Yang Ying, Gong Zhihua, Lian Hongyun, Ma Honghao, Liu Yali, Nie Xiaolu, Wang Dong, Zhang Li, Wei Ang, Cui Lei, Zhang Liping, Ou Wenxin, Wang Yizhuo, Wu Wanshui, Wang Tianyou, Li Zhigang, Zhang Rui
Аннотация
BACKGROUND: A portion of children with Langerhans cell histiocytosis (LCH) exhibit recurrent or persistent disease-related fever at their first visit. We aimed to clarify the clinical characteristics, laboratory indicators, prognostic differences, and optimal regimens for these children with fever.
METHODS: A cross-sectional design (single-center, retrospective, and observational) was adopted for this study. A total of 448 pediatric patients diagnosed with LCH in our hospital from January 2016 to December 2019 were retrospectively enrolled and divided into two groups: children with fever (n=52) and children without fever (n=396). The clinical characteristics, laboratory indicators, and outcomes of regimens for these children were analyzed with SPSS 16.0 software.
RESULTS: Children with initial fever had distinct clinical characteristics and laboratory indicators, including an age younger than two years, risk-organ involvement, accompanying hemophagocytic syndrome, and higher levels of cell-free DNA BRAF V600E mutation, among others. Patients in the fever group also had a significantly lower treatment efficacy from first-line treatment and a lower 3-year progression-free survival (PFS) rate (approximately 5.22% and 64.23%, respectively; P<0.001). Multivariate Cox regression analysis indicated that fever, risk-organ involvement, skin involvement, ferritin level, and dynamic erythrocyte sedimentation rate were independently prognostic factors for outcomes of first-line treatment. However, the 3-year PFS rates did not differ significantly between patients with fever and without fever after the administration of second-line or target regimens.
CONCLUSIONS: Among pediatric patients with LCH, those with initial fever exhibit more severe clinical symptoms and worse prognoses after first-line chemotherapy. Alternative strategies may mitigate fever-associated risk, and this possibility should be confirmed through prospective research.
METHODS: A cross-sectional design (single-center, retrospective, and observational) was adopted for this study. A total of 448 pediatric patients diagnosed with LCH in our hospital from January 2016 to December 2019 were retrospectively enrolled and divided into two groups: children with fever (n=52) and children without fever (n=396). The clinical characteristics, laboratory indicators, and outcomes of regimens for these children were analyzed with SPSS 16.0 software.
RESULTS: Children with initial fever had distinct clinical characteristics and laboratory indicators, including an age younger than two years, risk-organ involvement, accompanying hemophagocytic syndrome, and higher levels of cell-free DNA BRAF V600E mutation, among others. Patients in the fever group also had a significantly lower treatment efficacy from first-line treatment and a lower 3-year progression-free survival (PFS) rate (approximately 5.22% and 64.23%, respectively; P<0.001). Multivariate Cox regression analysis indicated that fever, risk-organ involvement, skin involvement, ferritin level, and dynamic erythrocyte sedimentation rate were independently prognostic factors for outcomes of first-line treatment. However, the 3-year PFS rates did not differ significantly between patients with fever and without fever after the administration of second-line or target regimens.
CONCLUSIONS: Among pediatric patients with LCH, those with initial fever exhibit more severe clinical symptoms and worse prognoses after first-line chemotherapy. Alternative strategies may mitigate fever-associated risk, and this possibility should be confirmed through prospective research.