{"entity":{"id":"paper-rosai-dorfman-destombes-consensus-recommendations-blood-2018","kind":"paper","name":"Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease","aka":[],"tldr":"The first expert guidance on Rosai-Dorfman disease: how to confirm the diagnosis, which patients can be watched, and which need surgery, steroids, chemotherapy or targeted treatment.","summary":"Consensus from the Histiocyte Society and international experts on Rosai-Dorfman-Destombes disease covering histopathology (S100 and CD68 positive, CD1a negative histiocytes with emperipolesis), the clinical spectrum from isolated lymph node disease to extranodal, cutaneous and IgG4-related forms, molecular findings (KRAS and MAP2K1 mutations in a subset), and management: observation for asymptomatic disease, surgery for isolated lesions, corticosteroids, sirolimus, cladribine or other chemotherapy, and MEK inhibitors for MAPK-mutant disease.","asOf":"2026-09-18","links":[{"label":"Blood 2018","url":"https://doi.org/10.1182/blood-2018-03-839753"},{"label":"PubMed","url":"https://pubmed.ncbi.nlm.nih.gov/29720485/"}],"tags":[],"related":[],"cancers":["rosai-dorfman-disease"],"sections":[],"technologies":[],"targets":[],"drugs":["cobimetinib","cladribine"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":["blood"],"dependsOn":[],"notes":[],"journal":"Blood","year":2018,"doi":"10.1182/blood-2018-03-839753","pmid":"29720485","authors":"Abla O, Jacobsen E, Picarsic J, et al.","paperType":"guideline","findings":[],"whatItMeans":"The treatment rows on the Rosai-Dorfman page, from watchful waiting to cobimetinib, follow these recommendations.","caveats":["Consensus on case series; no prospective trial exists.","The proportion of cases that are clonal neoplasms versus reactive is still debated."],"changedPractice":true},"route":"/key-papers/paper-rosai-dorfman-destombes-consensus-recommendations-blood-2018/","neighbours":{"cancer":[{"id":"rosai-dorfman-disease","kind":"cancer","name":"Rosai-Dorfman-Destombes disease","route":"/cancers/rosai-dorfman-disease/"}],"drug":[{"id":"cladribine","kind":"drug","name":"Cladribine","route":"/drugs/cladribine/"},{"id":"cobimetinib","kind":"drug","name":"Cobimetinib","route":"/drugs/cobimetinib/"}],"journal":[{"id":"blood","kind":"journal","name":"Blood","route":"/journals/blood/"}]}}