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Langerhans cell histiocytosis in children less than 2 years of age
P Kusuma Kumary1, T Priyakumari, V G Chellam
1Division of Pediatric Oncology and Radiation Oncology, Regional Cancer Center, Trivandrum, Kerala, India.
Insights
Langerhans cell histiocytosis (LCH) in young children without organ dysfunction has a good prognosis and requires less aggressive treatment. However, LCH with organ dysfunction in this age group is fatal, necessitating intensive chemotherapy.
Area of Science:
- Pediatric Oncology
- Hematology
- Immunology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder of Langerhans-like cells.
- Early diagnosis and appropriate management are crucial for improving outcomes in pediatric LCH.
Purpose of the Study:
- To investigate the clinical characteristics and treatment outcomes of LCH in children under two years of age.
- To identify prognostic factors differentiating outcomes in this pediatric population.
Main Methods:
- Retrospective analysis of medical records for children diagnosed with LCH up to two years of age.
- Patients were stratified into groups based on the presence or absence of organ dysfunction.
- Treatment modalities included surgery, radiotherapy, and chemotherapy, tailored to disease extent.
Main Results:
- Twenty children diagnosed with LCH under two years of age were analyzed over a 12-year period.
- Thirteen patients lacked organ dysfunction, while seven presented with organ dysfunction.
- All children with organ dysfunction succumbed to the disease, whereas 11 of 13 without organ dysfunction achieved disease-free survival with treatment.
Conclusions:
- Pediatric LCH without organ dysfunction indicates a favorable prognosis, suggesting conservative treatment approaches.
- LCH with organ dysfunction in young children is associated with a poor prognosis, mandating aggressive multi-agent chemotherapy.
Objective:
To study the clinical profile and outcome of langerhans cell histiocytosis in children upto 2 years of life.
Design:
Retrospective analysis.
Methods:
Medical records of Children upto 2 years of age with a diagnosis of langerhans cell histiocytosis (LCH) were analyzed. Their clinical pattern, treatment modalities and outcome were studied. The patients Were categorized into 2 groups according to their clinical presentation: (i) Subject without organ dysfunction; and (ii) cases with organ dysfunction. Treatment considered of surgical intervention, radiotherapy, chemotherapy or combination of all these modalities depending upto the extent of disease.
Results:
There were 20 children upto 2 years of age with histiocytosis during the 12 year period (January 1983 - December 1994). The median age at diagnosis was 18 months (range 52 days - 24 months). Of the twenty patients,13 patients didn't have organ dysfunction and 7 had organ dysfunction. Out of the 13 children without organ dysfunction eleven patients received treatment and all of them are alive free of disease with a median follow up of 62 months. But all children with organ dysfunction succumbed to disease within a few weeks.
Conclusion:
Children under 2 years of age with localised and or multifocal LCH without organ dysfunction have a good prognosis and they should not be exposed to aggressive form of treatment. All children with organ dysfunction require multi-agent chemotherapy.
