Abstract
PurposeThis study determined the frequency of clinical features, reactivations, sequelae, mortality, and overall survival (OS) and compared paediatric with adult Langerhans cell histiocytosis (LCH) patients.Materials and methodsNinety patients (60 paediatric and 30 adults) with LCH treated during 28 years were analysed retrospectively.ResultsCraniofacial lesion was the most frequent lesion at LCH presentation in children and adults. However, some differences were found. Orbital lesions were more frequent in paediatric than adult patients (P = 0.001). There was a tendency for mandible lesions to be more common in adults than the paediatric group (P = 0.0710). Mucocutaneous lesions were observed in a higher proportion in adults compared to paediatric patients (P = 0.0395). Reactivation episodes (36.8 versus 62.5%) and deaths (10.7 versus 24.0%) occurred in lower proportions in paediatric than adult patients, respectively. The probability of OS in 10 years for both groups was similar (P = 0.137).ConclusionThe OS was similar in both groups despite clinical differences between paediatric and adult patients, and higher reactivation and death rates in adults.
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