This protocol covers adult patients presenting with multifocal cutaneous Langerhans cell histiocytosis — skin-only disease characterised by involvement of multiple cutaneous lesion sites. Treatment recommendations in this setting take into account disease extent and the adequacy of prior local or systemic options.
When systemic intervention is indicated for multifocal cutaneous LCH in adults, the protocol specifies a systemic chemotherapy regimen. The agents selected, cycle structure, and full dosing details are available in the complete protocol.
Response is assessed using FDG-PET-based imaging after approximately 2–3 months of treatment initiation. Subsequent imaging frequency is tailored individually based on the clinical picture.
DOI: 10.1182/blood.2021014343
For multifocal cutaneous LCH, recommended treatments are topical therapy, oral low-dose weekly methotrexate ± prednisone/6-MP, hydroxyurea, or IMiDs.
For disease recurrence not amenable to local therapies or unresponsive to immunosuppressive agents, systemic chemotherapy is 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.
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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