Clinical profile of Langerhans Cell Histiocytosis at a tertiary centre: a prospective study

To study the varied presentations of Langerhans Cell Histiocytosis (LCH), the differential diagnosis of the varied presentations and the time lag in achieving the diagnosis. Prospective analysis of children diagnosed to have LCH over a period of 51 mo was done. A complete history and physical examination was undertaken in all patients, followed by relevant laboratory and radiological evaluation. Biopsy of the appropriate specimen was done. The extent of the disease was documented, accordingly treated and followed up.

There were 16 children with LCH from October 2005 through December 2009. The age ranged from 8 mo to 72 mo. Diagnosis was confirmed by CD1a/S 100 in 15 children (93.75%). The mean time to arrive at the diagnosis was 9.9 mo. Multisystem disease was documented in 11 (68.75%) children and there were 4 (25.0%) cases of pulmonary LCH. The mean time of follow-up was 14.4 mo (range, 1 mo to 50.6 mo). Most common referral diagnoses in LCH patients was recurrent pneumonia and immunodeficiency.

There is a need for high index of suspicion for diagnosis of LCH; misdiagnosis is frequent. Pulmonary involvement in children with LCH appears common. It is possibly still underdiagnosed. Nail changes are uncommon, but may act as a marker for multisystem disease. In addition to survival data and analysis of prognostic factors, the prospective collection of data on diverse presentations is essential, along with a high index of suspicion for the diagnosis of LCH.