Clinical analysis of 112 patients with single-system Langerhans cell histiocytosis

Abstract

Objective To investigate the clinical characteristics, treatment and prognosis of single- system Langerhans cell histiocytosis (LCH) in children. Methods A retrospective analysis was performed in single- system LCH patients registered between January 2006 and December 2012 in Beijing Children's Hospital Affiliated to Capital Medical University.The patients were divided into 2 groups: the bone involvement group and the other organ involvement group.The patients were assessed at 5 weeks, 11 weeks, 25 weeks, 3 months, 6 months, 1 year and 3 years.The data were analyzed by using SPSS 17.0 software. Results A total of 112 patients (66 boys and 46 girls) with a me-dian age of 5 years at diagnosis of LCH were analyzed.The most frequently affected organ was the bones(91 cases, 81.3%), followed by skin(15 cases, 13.4%). Few patients (27.6%) had acentral nervous system risklesion, who were younger than those with other bone lesion(2.5 years vs 6.6 years). Patients with bone lesions were diagnosed at a significantly older age than other patients(5.6 years vs 1.5 years)(P<0.01). All patients received chemotherapy that included Prednisone and Vinblastine for 25 weeks.Twenty-five patients (22.3%) showed reactivation.Of these, 4 patients exhibited reactivation in the pituitary.Three-year overall survival rate was expected to reach 100%, and no-event survival was expected at (73.22±4.47)%.Age of less than 2 years old was the factor of reactivation(P=0.033); sex, organ involvement and member of bone involved were not related with reactivation(P=0.679, 0.142, 0.639). Conclusions The bones were the frequent involvement organ in single-system LCH patients.These patients have a good prognosis.The rate of reactivation of single system-LCH can be decreased by chemotherapy. Key words: Langerhans cell histiocytosis; Single system; Treatment