Significance of immunological system examinations in the clinical course of Langerhans cell histiocytosis in children

: The Langerhans cell histiocytosis (LCH) is an enigmatic disease, usually occurring in the young. Its etiology is unknown, its pathogenesis is not correctly understood and the clinical course is unpredictable. In the years from 1975 to 1998 in Department of Paediatric Haematology and Oncology (Wroclaw University School of Medicine) 32 children with diagnosis of the Langerhans Cell Histiocytosis (LCH) were treated. The study group included 19 boys and 13 girls, aged from 3 weeks to 14 years, median age 7.5 years. Investigations of immunological system were performed in the examined group. In all children lymphocytes' subsets were examined. We examined apoptosis with ELISA test in 3 patients, in 3--marked antigen CD44, in 12 marked sVCAM-1 by ELISA method. IL-1 in growing cell supernatants was determined in 17 children. In 15 children lower IL-1 production was observed before the beginning of therapy in comparison with control group. In all patients decreased concentrations of lymphocytes T were noted, but in 8 children lower CD4/CD8 rate was observed. In the study group higher expression of CD44 was noted. In 3 patients cell apoptosis was normal. In 12 patients lower expression of sVCAM-1 was observed. Abnormal expression of adhesion molecules may influence the pathogenesis of LCH. Disorders of immunological system were observed in the patients with LCH.