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[Treatment results of Langerhans cell histiocytosis with LSH II protocol]
H Mottl1, M Ganevová, J Radvanská
1Klinika dĕtské hematologie a onkologie 2. LF UK a FNM, Praha. hubert.mottl@fnmotol.cz
Insights
The LCH II protocol is a safe and effective treatment for Langerhans cell histiocytosis in children. Modifications may be needed for multisystem disease cases.
Area of Science:
- Pediatric Oncology
- Hematology
- Clinical Trials
Background:
- Langerhans cell histiocytosis (LCH) is a rare disorder affecting children.
- International treatment protocols aim to standardize and improve LCH management.
- Evaluating treatment outcomes is crucial for refining therapeutic strategies.
Purpose of the Study:
- To assess the effectiveness and safety of the international LCH II treatment protocol.
- To analyze outcomes for children with Langerhans cell histiocytosis treated at FN Motol.
- To identify potential areas for treatment modification, particularly for multisystem disease.
Main Methods:
- Retrospective analysis of 46 children diagnosed with LCH between 1995 and 2003.
- Application of the LCH II protocol, including low-risk (LR) and high-risk (HR) arms.
- Use of chemotherapy agents such as etoposide and 2-chlorodeoxyadenosine (CDA) for relapsed cases.
Main Results:
- Overall, 63.0% of children achieved first complete remission (CR), 30.4% achieved second CR, and 4.4% achieved third CR.
- Monosystem disease, predominantly bone lesions, was observed in 60.9% of patients, with surgery as primary treatment.
- Multisystem disease affected 39.1% of patients; 10 out of 11 relapsed cases achieved second or third CR with CDA monotherapy.
Conclusions:
- The LCH II protocol demonstrates safety and efficacy in treating pediatric Langerhans cell histiocytosis.
- Treatment strategies for multisystem LCH may require adjustments to optimize outcomes.
- Further research into modified protocols for complex LCH cases is warranted.
Background:
The aim of study was to evaluate outcome of international treatment protocol LCH II for children with Langerhans cell histiocytosis treated in FN Motol.
Methods And Results:
Between November 1995 and December 2003, 46 children were treated, sex ratio M:F 29:17 and median age at diagnosis 6 years 8 months. 28 children (60.9%) suffered from monosystem disease with majority of bone lesions (23 times) with skull predominance (16 times). Surgery was primary treatment modality for monosystem disease. Five children with recurrence were successfully treated by protocol LCH II - LR (3x) and LCH III - LR /G2/, respectively. Eighteen children (39.1%) suffered from multisystem disease. 6 out of 18 patients were treated according to low-risk protocol LCH II - LR and 12 children by high-risk scheme LCH II - HR at the non-randomized branch included etoposide. Recurrence was revealed in 11 patients and 10 of them reached 2nd or 3rd complete remission (CR) by 2 - chlorodeoxyadenosine (CDA) monotherapy, and 1 child reached 2nd CR by LCH II - HR scheme. Two children underwent irradiation after bone lesion excision as well as 1 child as supplemental treatment. Totally, 29 children (63.0%) achieved 1st CR, 14 (30.4%) 2nd CR, 2 (4.4%) 3rd CR, and 1 child died because of LCH progression. There were no severe side effects of chemotherapy. Follow-up median time was 5 years 8 months (range 9 months - 9 years 6 months).
Conclusions:
LCH II protocol is safe and effective. Results revealed that treatment of patients with multisystem disease might demand some treatment modification.
