Langerhan's cell histiocytosis: A single institutional experience.

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.