Erdheim-Chester disease: 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 | Biopsy of an accessible lesion with BRAF V600E testing and broader sequencing; FDG-PET/CT, cardiac and brain MRI, endocrine assessment. | Consensus recommendations for Erdheim-Chester disease (Blood 2020) | 87 |
Second line
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Second-choice or intolerant | Interferon alfa or pegylated interferon, anakinra, cladribine, methotrexate. | Consensus recommendations for Erdheim-Chester disease (Blood 2020) | 78 |
Other settings
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Asymptomatic bone-limited disease | Observation with periodic PET and organ screening. | Consensus recommendations for Erdheim-Chester disease (Blood 2020) | 66 | |
| BRAF | BRAF V600E-mutant symptomatic disease | Vemurafenib (approved 2017) or dabrafenib with trametinib; cobimetinib as an alternative; dose reduction to limit toxicity, treatment continued long term. | Consensus recommendations for Erdheim-Chester disease (Blood 2020) | 97 | |
| BRAF | BRAF wild-type symptomatic disease | Cobimetinib (approved 2022 for histiocytic neoplasms); trametinib as an alternative MEK inhibitor. | Consensus recommendations for Erdheim-Chester disease (Blood 2020) | 97 |
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.