Treatment of Multisystem Langerhans Cell Histiocytosis in Adults
Adults presenting with multisystem Langerhans cell histiocytosis — defined as involvement of two or more organ systems — or with extensive or refractory multifocal single-system LCH require a systemic treatment strategy. This is a first-line protocol addressing that clinical scenario.
Clinical Scenario
Multisystem LCH with involvement of two or more organ systems, or extensive/refractory multifocal single-system LCH, in an adult patient. The extent of disease places this beyond approaches suitable for localised single-site involvement.
Treatment Approach
Systemic chemotherapy is the basis of management, with the full protocol specifying both a preferred regimen and a recognized alternative — complete agent selection, sequencing, and all dosing remain in the structured protocol.
Clinical Goal
Disease response assessed by FDG-PET-based imaging after 2–3 months of therapy.
References
DOI: 10.1182/blood.2021014343
- Multisystem | ≥2 organ/system involvement.
- For multisystem LCH or extensive/refractory multifocal single-system LCH, systemic chemotherapy agents such as cladribine, cytarabine, or vinblastine + prednisone are recommended.
- Among systemic treatment options for LCH, chemotherapy using either cladribine or cytarabine is preferred because of relatively high overall response rates and the potential for long-term remissions with limited cycles of treatment.
- Vinblastine + prednisone is a reasonable alternative, although it is not the preferred choice of therapy in adults given the high risk of relapse and potential for peripheral neuropathy.
- For initially FDG PET avid LCH, it is recommended to repeat an FDG PET-based imaging study for assessment of disease response after 2-3 mo of initiation of therapy, with subsequent imaging frequency tailored individually based on the specific clinical scenario.
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