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Langerhans Cell Histiocytosis (LCH) in Egyptian Children: Does Reactivation Affect the Outcome?
Mohamed Sedky Mahmoud Sedky1,2, Hany Abdel Rahman3,4, Emad Moussa4,5
1Department of Pediatrics, National Research Centre, Cairo, Egypt. mohamed.sedky@57357.com.
Insights
This study reports on pediatric Langerhans cell histiocytosis (LCH) management in Egypt. Treatment showed good initial response and survival, though high-risk cases require new therapies.
Area of Science:
- Pediatric Oncology
- Hematology
- Immunology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal disorder affecting multiple organ systems in children.
- Effective management strategies are crucial for improving outcomes in pediatric LCH patients.
Purpose of the Study:
- To evaluate the single-center outcomes of pediatric Langerhans cell histiocytosis (LCH) management.
- To assess treatment response, survival rates, and identify challenges in a specific patient cohort.
Main Methods:
- Retrospective analysis of 80 pediatric LCH patients treated between July 2007 and December 2011.
- Patients were stratified and managed according to the LCH III protocol.
- Median follow-up was 42 months.
Main Results:
- Initial treatment response at 6 and 12 weeks was high (76% and 93%).
- Disease reactivation occurred in 38% of patients, with 6.25% experiencing multiple episodes.
- Five-year overall survival (OS) was 96.3%, and event-free survival (EFS) was 55%.
Conclusions:
- Pediatric LCH management in this Egyptian cohort demonstrated satisfactory treatment response and survival.
- High-risk organ disease necessitates novel therapeutic agents for salvage.
- Repetition of first-line treatment is effective for managing multiple LCH reactivations.
Objective:
To report a single centre outcome of management of Langerhans cell histiocytosis (LCH), a clonal disease with involvement of various body systems.
Methods:
Retrospective analysis of 80 pediatric LCH patients at Children Cancer Hospital-Egypt between July 2007 and December 2011 was performed. Patients were stratified and treated according to LCH III protocol. The median follow up period was 42 mo (range: 1.18 to 71 mo).
Results:
At wk 6 and 12, 'better' response was obtained in 61 (76 %) and 74 (93 %) patients respectively. Afterwards, reactivation occurred in 25 patients (38 %), of them multiple episodes occurred in 5 patients (6.25 %), managed by repetition of 1st line treatment for once or more. The 5 y overall survival (OS) and event free survival (EFS) was 96.3 and 55 % respectively. At last follow up, better status was reached in 70 patients, 3 in each 'intermediate' and 'worse' status. Three high risk patients died and one patient was lost to follow up.
Conclusions:
In a single Egyptian pediatric LCH experience, the response to treatment is satisfactory and survival remains the rule except in high risk organs disease that still needs a new molecule for salvage. However in multiple reactivations, patients do well with repetition of the 1st line of treatment with or without methotrexate.
