The Turkish Journal of Pediatrics 2009; 51: 344-349 Original Langerhans cell histiocytosis in Turkish children

Langerhans cell histiocytosis (LCH) is a rare disorder with diverse forms of clinical presentation ranging from a benign course to diffuse progressive disease, and descriptions of LCH generally have been limited to single case reports and small case series. Since the condition is uncommon, we aimed in this study to describe our own experience and discuss the findings, treatment and outcome in our patients with LCH in light of the current literature. The eight patients (5 boys, 3 girls) ranged in age from 2 months to 3 years (mean 18±10.8 months). Multiple organ involvement was noted in 3, isolated bone involvement in 3, orbital involvement in 1, and pituitary gland involvement with rash in 1 patient. Treatment modalities used varied from simple observation to chemotherapy. Outcome results of our study demonstrated resolution of lesions in 5 patients and death in 2 patients. One patient with pituitary gland involvement developed diabetes insipidus and is receiving intranasal desmopressin acetate