Abstract
Background:Langerhans cell histiocytosis (LCH) is a disease that primarily affects bone but can be associated with a clinical spectrum that ranges from a solitary bone lesion with a favorable natural history to a multisystem, life-threatening disease process.Aim:We analyzed our single institutional experience of managing children with LCH.Settings and Design:A total of 40 children of LCH, managed in tertiary cancer center in South India in the period from 2001 to 2005, were evaluated retrospectively.Materials and Methods:Clinicopathological features, laboratory findings, treatment modalities and long-term outcome were analyzed.Results:Children were aged between 2 months and 12 years, with a mean of 3 years. Majority of the children were below 5 years of age. Group B constituted a bulk of children. Disseminated cases were less (five patients). Liver function dysfunction was seen in four (10%) children. Pulmonary interstitial infiltrates were seen in two (5%) cases. Diabetes insipidus manifested in three patients. There was one death.Conclusion:A better understanding of the etiology and pathogenesis of LCH will result in more directed and efficacious treatment regimens.
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