ABSTRACT
BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disease with abnormal accumulation of the dendritic Langerhans cells. In the localized form (single system), the disease is self-limiting but in the cases of multisystem disease, one third of the patients develop organ dysfunction with poor prognosis. The aim of this study was to examine the role of p53 and vascular endothelial growth factor (VEGF) in the pathogenesis of LCH and look for association of them with the extent of the disease. MATERIALS AND METHODS: Biopsy specimens obtained from 26 patients with definitive diagnosis of LCH were stained immunohistochemically for p53 and VEGF. RESULTS: There were 13 male and 13 female cases. The mean age of patients at presentation was 41.9 months (range, 2 mo to 18 y). Multisystem disease was presented by 61% of the patients (8 boys and 8 girls). Patients with multisystem disease were on average older than those with single system disease. p53 protein could be detected in 92% of cases and 61.5% of patients expressed VEGF, mostly from multisystem group. CONCLUSIONS: These findings highlight the role of angiogenic factors in the clinical behavior of LCH and might be of prognostic or therapeutic importance. However, further studies, with larger sample sizes are warranted.
