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. Based on our results and a review of the literature, we recommend that any child with suspected solitary LCH undergo a full diagnostic investigation to rule out multiple lesions. A biopsy is recommended for a diagnosis at the time of presentation and should be attempted in any suspicious lesion. Chemotherapy is reserved for multiple systemic lesions and central nervous system (CNS) risk lesions. Follow-up investigations should be individualized but should consist of radiography and magnetic resonance imaging (MRI). Studies suggest that follow-up of at least four years is required.