Case Study on Langerhans Cell Histiocytosis of Bone

Abstract

Aims: To precise, the epidemiological, clinical, para-clinical, therapeutic and prognostic characteristics of skeletal involvement in Langerhans cell histiocytosis Materials and Methods: A retrospective and descriptive study of patients with Langerhans cell histiocytosis admitted in Internal Medicine Departments of Hedi Chaker University Hospital of Sfax between 1996 and 2018. Cases of Langerhans cell histiocytois confirmed with histo- pathological examination were included. Results: Four cases of LCH with bone involvement were noted. All patients enrolled were male and the mean age at diagnosis was 23.25 years. The bone lesions were unifocal in two cases and multifocal with multisystemic LCH in the others. The treatment consisted of curettage in two cases and two patients received systemic therapy with corticosteroids and vinblastine respectively. The outcome was favorable in two patients with eosinophilic ganuloma and systemic replases were noted with novel bone lesions in two patients presenting the systemic form of the disease. Conclusion: LCH is a rare disease in children and young adult males. In the present series, bone was the most frequently involved site. The circumstances of discovery of bone localization were the pain swelling lesion in different sites. Biopsy is necessary to obtain diagnosis confirmation. The prognosis of this pathology depends largely on early diagnosis, other organs affected and the response to treatment. The new class of BRAF inhibitors may be a promising therapeutic option in LCH which needs to be assessed in prospective studies mainly in bone lesions.