OnCo

Langerhans cell histiocytosis (LCH): lines of therapy

Cancer page →

4 standard-of-care settings across 3 lines and 2 biomarker subgroups. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.

LineAll comersFRα
Advanced, first line1·
Third line and beyond·1
Other settings2·

Advanced, first line

SubgroupSettingApproachProducts and trialsEvidence
All comersMultisystem LCH (first line)Vinblastine and prednisone for 12 months (LCH-III), with response assessment at 6 weeks; LCH-IV tests further tailoring of duration and intensity.81

Third line and beyond

SubgroupSettingApproachProducts and trialsEvidence
FRαRefractory risk-organ disease or reactivationCladribine plus cytarabine or clofarabine salvage; BRAF inhibitor (vemurafenib or dabrafenib, with trametinib) for BRAF V600E, MEK inhibitor for MAP2K1-mutant disease.84

Other settings

SubgroupSettingApproachProducts and trialsEvidence
All comersSingle-system bone or skinBiopsy or curettage with observation; intralesional steroid, topical therapy or indomethacin for symptomatic lesions; systemic therapy for multifocal bone or CNS-risk lesions.75
All comersNeurodegenerative LCHMAPK-pathway inhibition (BRAF or MEK inhibitor) with neurological monitoring; early MRI detection in children with pituitary or craniofacial disease.84

Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.