Predictors of outcome in children with Langerhan's cell histiocytosis

Abstract

8550 Background:Analysis of the clinical course of patients with Langerhans cell histiocytosis(LCH). Methods:We conducted a retrospective chart review of 132 patients treated at Childrens Hospital Los Angeles for LCH from 1984 to 2001. Diagnosis was confirmed histologically in all cases. Age at first diagnosis, number of bone lesions involved, organ involvement, and outcomes after initial therapy were evaluated. Patients were divided into three groups: Patients with single bone lesions at presentation, patients with multiple bone lesions at presentation and patients with multiple organ involvement. Results:The mean age at presentation was five years in patients with bone disease only as compared with two years in patients with other organs involved. Patients were either treated with surgical curettage, chemotherapy, and or external beam radiation. The risk for reactivation after initial management is significantly higher for patients with multiple bone and those with multiple organ involvement as compared with patients who had a single lesion at presentation. Hazard raios are 7.1 and 11.6 respectively. However the risk of death or progression is highest in patients with multiple organ involvement. Patients younger than one year of age in the multiple organ group have a significant increased risk of death at two years when compared with older patients in that group (hazard ratio=6.2, p=0.022). Endocrine abnormalities due to pituitary dysfunction were seen in 20% and 7.5% of the patients with or without skull lesions respectively. In addition, more patients with multiple organ involvement developed endocrine abnormalities when compared with the other two groups (p0.001). Conclusions: Patients with LCH involving only the bones have a significantly better outcome than those with multiple organ involvement. However, the rate of reactivation after initial therapy is significantly higher for patients with multiple bone involvement as compared with patients in the single bone lesion group. Patients with multiple organ involvement who are less than one year of age are at particular high risk of death and should be approached more aggressively upfront. No significant financial relationships to disclose.