This protocol covers the first-line management of Langerhans cell histiocytosis (LCH) when disease involves the brain parenchyma (CNS) in an adult patient — a presentation that requires specific systemic treatment.
Langerhans cell histiocytosis with brain parenchymal (CNS) involvement in an adult. CNS involvement defines a distinct disease distribution that directly shapes which treatments are recommended at first line.
First-line management for this scenario centres on systemic chemotherapy; the specific agents considered appropriate for CNS involvement, and additional preferred options, are detailed in the full protocol.
Disease response on FDG-PET-based imaging after 2–3 months of therapy.
DOI: 10.1182/blood.2021014343
For LCH involving the brain parenchyma, first-line treatment with chemotherapy with cladribine or cytarabine is recommended.
For patients with CNS disease, cladribine, higher doses of cytarabine, IV methotrexate-based regimens, or kinase inhibitors are preferred.
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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