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Published on: October 19, 2014
Langerhan's cell histiocytosis: A single institutional experience
Tejinder Singh1, C T Satheesh, L Appaji
1Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore - 560 030, India.
Summary
Langerhans cell histiocytosis (LCH) in children can range from solitary bone lesions to multisystem disease. This study analyzed 40 South Indian children, finding most cases were not disseminated, with few severe complications.
Area of Science:
- Pediatric Oncology
- Hematology-Oncology
- Histiocytosis
Background:
- Langerhans cell histiocytosis (LCH) is a rare disorder primarily affecting bone but with potential for multisystemic involvement.
- LCH presents a spectrum from localized bone lesions to life-threatening disseminated disease.
Purpose of the Study:
- To retrospectively analyze the clinical experience of managing pediatric LCH cases at a tertiary cancer center in South India.
- To evaluate clinicopathological features, treatment strategies, and outcomes in children with LCH.
Main Methods:
- Retrospective analysis of 40 children diagnosed with LCH between 2001 and 2005.
- Data collected included clinicopathological characteristics, laboratory results, treatment modalities, and long-term outcomes.
Main Results:
- The study included 40 children aged 2 months to 12 years (mean 3 years), with most below 5 years.
- Disseminated LCH was infrequent (5 patients); significant complications included liver dysfunction (10%), pulmonary infiltrates (5%), and diabetes insipidus (3 patients).
- One death was recorded during the study period.
Conclusions:
- Understanding LCH etiology and pathogenesis is crucial for developing targeted and effective treatments.
- This institutional experience highlights the varied presentation and outcomes of pediatric LCH.