# Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease

Source: https://onco.cc/key-papers/paper-rosai-dorfman-destombes-consensus-recommendations-blood-2018/  
OnCo record `paper-rosai-dorfman-destombes-consensus-recommendations-blood-2018` (Key paper). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Fields

- Kind: Key paper
- Last checked: 2026-09-18
- Journal: Blood
- Year: 2018
- DOI: 10.1182/blood-2018-03-839753
- Authors: Abla O, Jacobsen E, Picarsic J, et al.
- What it means: 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.

## Sources

- Blood 2018: https://doi.org/10.1182/blood-2018-03-839753
- PubMed: https://pubmed.ncbi.nlm.nih.gov/29720485/

## Connected records

- cancers: [Rosai-Dorfman-Destombes disease](https://onco.cc/cancers/rosai-dorfman-disease/)
- drugs: [Cladribine](https://onco.cc/drugs/cladribine/), [Cobimetinib](https://onco.cc/drugs/cobimetinib/)
- journals: [Blood](https://onco.cc/journals/blood/)

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