Clinical Profile of Langerhand Cell Histiocytosis- Single Center Experience

Background/Objectives:

Dubai hospital is one of the three main centers which carters for the management of children presenting with benign and malignant blood disorders in the United Arab Emirates. This study is to demonstrate the varied clinical profile of children with Langerhans cell histiocytosis (LCH) who presented to Dubai hospital since 2012.

Methods/Design:

A Prospective analysis of children with LCH treated at our center was done. Complete history, physical examination, relevant laboratory and radiological investigations were studied for all children suspicious to have LCH. Diagnosis was confirmed by obtaining tissue from biopsy of involved areas. Once histopathology confirmed the diagnosis, extent of disease was evaluated and chemotherapy started based on the LCH-IV protocol.

Results:

A total of 10 children presented to Dubai hospital with LCH from 2012 to 2016. The age at diagnosis ranged from 5 months to 5 years. Diagnosis was confirmed by positive CD1a and S100 immuno-markers of involved tissue (100%). These children had varied presentations like respiratory distress, recurrent chest infections, chronic diarrhea, abdominal pain, back pain, inability to walk, lymphadenopathy and buccal mucosa swelling. Median time of diagnosis once the children reached to our center was between 15 days to 1 month. Five children (50%) had multisystem LCH with risk organ involvement. Four children (40 %) had single system involvement and one child had single system multifocal involvement. Osteolytic lesions were present in six children (60%) leading to an early diagnosis.

Good response to Induction I therapy (Prednisolone, Vinblastinefor 6 weeks) of Stratum I, LCH-IV protocol was seen in sevenchildren (70%). Two children were upgraded to Stratum II chemotherapy, LCH-IV protocol at the end of Induction II of stratum I chemotherapy (total 12 weeks of prednisone and Vinblastine) because of either poor response or progressive disease. One child was started on salvage chemotherapy, (Cladiribine, Cytarabine) during Induction therapy because of no response. There has been no mortality in our cohort.

Conclusion:

There should be high index of suspicion for LCH. One needs to be well acquainted to varied presentations of LCH for quickerdiagnosis. Induction therapy with Prednisolone and Vinblastine is a good prognostic marker for children with LCH in our study. It should still be considered the first line therapy for all children with LCH requiring chemotherapy because of lower toxicity profile and good response in the majority. Poor response to Initial Induction of 6 weeks with Vinblastine and Prednisolonewas salvageable with second line therapy in our cohort.