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Langerhans' cell histiocytosis: experience from a single center
Deepak Bansal1, R K Marwaha, Amita Trehan
1Pediatric Hematology-Oncology Unit, Department of Pediatrics, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012, India.
Indian Pediatrics
|August 30, 2008
Summary
Langerhans cell histiocytosis (LCH) in children often presents as multisystem disease. Disseminated LCH with organ dysfunction significantly predicts a fatal outcome, while localized LCH generally has a favorable prognosis.
Area of Science:
- Pediatric Oncology
- Hematology
- Pathology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder of Langerhans cell precursors.
- Understanding the clinical spectrum and outcomes of LCH is crucial for effective management.
Purpose of the Study:
- To analyze the clinical profile, disease distribution, and outcomes of pediatric Langerhans cell histiocytosis.
- To identify predictors of mortality in children diagnosed with LCH.
Main Methods:
- Retrospective analysis of 69 children diagnosed with LCH between January 1986 and December 2004.
- Review of clinical data, disease involvement, treatment protocols, and patient outcomes.
Main Results:
- Multisystem LCH affected 69.6% of patients; hepatic dysfunction was observed in 36.2%, often indicated by elevated alkaline phosphatase.
- Localized LCH generally had a favorable outcome, whereas disseminated disease with organ dysfunction led to death in 54% of cases.
- Diabetes insipidus occurred in 17.4% of patients; portal hypertension was associated with a fatal outcome.
Conclusions:
- Disseminated LCH with organ dysfunction is a significant predictor of fatal outcomes.
- Treatment strategies varied based on disease extent, with localized disease responding to oral steroids and disseminated disease requiring combination chemotherapy.
- Early identification and management of organ dysfunction are critical for improving survival in pediatric LCH.