Abstract：Objective To investigate the clinical features and prognosis of children with Langerhans cell histiocytosis (LCH). Methods A retrospective analysis was performed for the clinical data of 34 children with newly diagnosed LCH. Results The 34 children had a median age of 14.5 months (range:22 d to 60 months). Of all 34 children, 23 were aged 0-2 years and 11 were aged > 2 years. There were 17 children in the high-risk group and 17 in the low-risk group. Thirty children received chemotherapy, and the 6-week and 12-month overall response rates were 67% (20/30) and 87% (26/30), respectively. The 3-year overall survival (OS) rate was 86%±6% and the 3-year event-free survival (EFS) rate was 64%±9%. Compared with the low-risk group, the high-risk group had significantly lower 6-week chemotherapy response rate (47% vs 87%; P < 0.05), 3-year OS rate (72%±12% vs 100%; P < 0.05), and 3-year EFS rate (46%±13% vs 82%±9%; P < 0.05). There was no significant difference in the 12-month chemotherapy response rate between the high-risk and low-risk groups (80% vs 93; P > 0.05). The high-risk group had a recurrence rate of 27% and a mortality rate of 27%. There were no recurrence or deaths in the low-risk group. Conclusions Children with LCH have a high overall survival rate, but the high-risk patient has a low 6-week response rate of induction chemotherapy and poor long-term prognosis.
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