Clinical analysis of 217 children with Langerhans cell histiocytosis / 中华实用儿科临床杂志

ABSTRACT

Objective To investigate the clinical characteristics,treatment,and prognostic factors for Langerhans cell histiocytosis (LCH) in children.Methods A retrospective review of patients diagnosed as LCH was carried out between January 2007 and December 2012 in Beijing Children's Hospital,Capital Medical University.Target patients were divided into multi-organ high-risk groups (Group Ⅰ),multi-organ low-risk groups (Group Ⅱ),single-organ groups (GroupⅢ),and the corresponding intensity of chemotherapy was given.SPSS 17.0 statistical software was used to analyze the findings.The correlations among the affected organ,the early treatment response and the prognosis were analyzed.Results A total of 217 patients were analyzed including 127 boys and 90 girls with ratio of 1.4 ∶ 1.0 and a median age of 36 months (ranged from 2 months to 14 years) on the diagnosis of LCH,and there were 132 cases (60.8％) in group Ⅰ,33 cases (15.3％) in group Ⅱ and 52 cases (23.9％) in group Ⅲ.The median age on diagnosis was 20 months in group Ⅰ,42 months in group Ⅱ and 72 months in group Ⅲ.The most frequently affected organ was the bone (176 cases,81.2％).Among 217 patients,55 cases (25.3％) had recurrence and 12 cases died.The rate of 3-year overall survival was expected to be 90.78％.The rate of 3-year event free survival was expected to be 76.5％.Myelosuppression,liver function damage and infection were the most common side effects due to chemotherapy with the percentages of 48.4％ (105 cases),24.0％ (52 cases) and 12.4％ (27 cases).Risk organs involvement and no-response to initial therapy (after 6 weeks) indicated a worse prognosis (x2 =10.60,12.84,P =0.017,0.001).Conclusions Incidence of LCH in boys is slightly higher than girls in children.Peak age at onset of LCH in children is 1-3 years old.Bone is the most frequent involved organ.Involvement of risk organs and no-response to initial therapy are key factors in determining worse prognosis.A rescue therapy should be introduced earlier in these patients.