{"entity":{"id":"paper-erdheim-chester-disease-consensus-recommendations-blood-2020","kind":"paper","name":"Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis and treatment in the molecular era","aka":[],"tldr":"International experts set out how to diagnose Erdheim-Chester disease, which scans and mutation tests to do, and when to use BRAF and MEK inhibitors, interferon or other drugs.","summary":"Consensus recommendations from an international group of histiocytosis specialists covering the clinical features, imaging (whole-body PET-CT, cardiac and brain MRI), biopsy and molecular testing (BRAF V600E and other MAPK pathway alterations), the decision to treat, and therapy: BRAF inhibitors for BRAF-mutant disease, MEK inhibitors for BRAF wild-type disease, interferon alfa and other options, with guidance on monitoring and long-term management.\n\nIt updates the 2014 recommendations for the targeted therapy era.","asOf":"2026-09-18","links":[{"label":"Blood 2020","url":"https://doi.org/10.1182/blood.2019003507"},{"label":"PubMed","url":"https://pubmed.ncbi.nlm.nih.gov/32187362/"}],"tags":[],"related":[],"cancers":["erdheim-chester-disease"],"sections":[],"technologies":[],"targets":[],"drugs":["vemurafenib","cobimetinib","interferon-alfa"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":["blood"],"dependsOn":[],"notes":[],"journal":"Blood","year":2020,"doi":"10.1182/blood.2019003507","pmid":"32187362","authors":"Goyal G, Heaney ML, Collin M, et al.","paperType":"guideline","findings":[],"whatItMeans":"The diagnostic and treatment rows on the Erdheim-Chester page follow these recommendations.","caveats":["Expert consensus in a disease with a few hundred reported cases; randomised evidence does not exist.","Optimal duration of targeted therapy remains unknown."],"changedPractice":true},"route":"/key-papers/paper-erdheim-chester-disease-consensus-recommendations-blood-2020/","neighbours":{"cancer":[{"id":"erdheim-chester-disease","kind":"cancer","name":"Erdheim-Chester disease","route":"/cancers/erdheim-chester-disease/"}],"drug":[{"id":"cobimetinib","kind":"drug","name":"Cobimetinib","route":"/drugs/cobimetinib/"},{"id":"interferon-alfa","kind":"drug","name":"Interferon alfa-2a/2b","route":"/drugs/interferon-alfa/"},{"id":"vemurafenib","kind":"drug","name":"Vemurafenib","route":"/drugs/vemurafenib/"}],"journal":[{"id":"blood","kind":"journal","name":"Blood","route":"/journals/blood/"}]}}