Single-system Langerhans cell histiocytosis (bone, skin or one other organ): lines of therapy
5 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.
Screening, prevention and diagnosis
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Diagnosis and staging | Biopsy with immunohistochemistry and BRAF testing; skeletal survey or whole-body imaging, blood count, liver tests and abdominal ultrasound to exclude multisystem disease. | Histiocyte Society evaluation and treatment guidelines; NCI PDQ | 95 |
Third line and beyond
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| FRα | Adult single-system or refractory disease | Cytarabine or cladribine; smoking cessation for pulmonary disease; BRAF or MEK inhibitors (vemurafenib, dabrafenib-trametinib, cobimetinib) for refractory disease. | Histiocyte Society evaluation and treatment guidelines; NCI PDQ | 97 |
Other settings
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Unifocal bone disease | Biopsy with curettage, with or without intralesional methylprednisolone; observation of healing; indomethacin for pain. | Histiocyte Society evaluation and treatment guidelines; NCI PDQ | 63 | |
| All comers | Multifocal bone or special-site disease | Vinblastine and prednisone for twelve months (LCH-III schedule) to reduce reactivation and central nervous system risk. | Histiocyte Society evaluation and treatment guidelines; NCI PDQ | 81 | |
| All comers | Skin-only disease | Observation or topical corticosteroids; systemic therapy only for extensive symptomatic disease; regular review for progression to multisystem disease. | Histiocyte Society evaluation and treatment guidelines; NCI PDQ | 63 |
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.