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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.
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.
The treatment rows on the Rosai-Dorfman page, from watchful waiting to cobimetinib, follow these recommendations.
Shares Rosai-Dorfman-Destombes disease, Blood.
Shares Cobimetinib, Blood.
Shares Cladribine, Rosai-Dorfman-Destombes disease, Cobimetinib.
Shares Cladribine, Rosai-Dorfman-Destombes disease, Cobimetinib.
Shares Cladribine, Rosai-Dorfman-Destombes disease, Cobimetinib.
Shares Cladribine, Cobimetinib.