Introduction Langerhans cell histiocytosis (LCH) is characterised by a reactive clonal proliferation and accumulation of dendritic cells with a wide range of clinical presentations.
Survival rate depend on single or multisystem disease.
Objectives The aim of the study was to analyse the clinical, radiologic features and responses to treatment.
Materials and methods We retrospectively reviewed the clinical data, histopathological, radiologic features, treatment modalities, and outcome of patients presenting with LCH.
Results 9 patients were included with two brothers. There were 5 girls and 5 boys. Mean Age at diagnosis was 39 months. The main clinical feature was prolonged ferver (5 cases), and impaired general condition (3 cases).
Skin involvement was present in 5 patients, otitis in 3 patients and 3 cases of lung injury with Spontaneous pneumothorax in one case. 3 different Tumour syndromes were observed at diagnosis. The most of patients present a multi-system disease.
Radiologic finding showed 2 cases of bone involvement. The bone marrow involvement was present in 2 patients. Six patients received corticosteroid and vinblastine combination with the use of cyclosporine in 3 cases. One patient developed insipidus diabetes. Two patients dead.
Conclusion Childhood Langherhans cell histiocytosis is a rare and poorly understood multi-system disease. Treatment decisions are difficult given the unpredictable course of the disease sometimes spontaneous, mainly for unifocal forms remissions.
Patients with localised disease generally have a good prognosis and require minimal treatment. However, patients with lesions in ‘risk’ organs (liver, spleen, lung, bone marrow) have a worse overall prognosis regarding mortality and morbidity.