Rosai-Dorfman-Destombes disease
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| Section editor | |
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Gaurav Goyal, MD UAB Birmingham, AL  GauravGoyalMD
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Guidelines
Consensus guidelines
- 2018: Abla et al. Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease PubMed
NCCN
All lines of therapy
Cobimetinib monotherapy
Regimen
| Study | Evidence |
|---|---|
| Diamond et al. 2019 (MSK 15-216) | Pilot, <20 pts in this arm |
Note: there were N=2 patients with this type of histiocytosis.
Targeted therapy
- Cobimetinib (Cotellic) 60 mg PO once per day on days 1 to 21
28-day cycles
References
- MSK 15-216: Diamond EL, Durham BH, Ulaner GA, Drill E, Buthorn J, Ki M, Bitner L, Cho H, Young RJ, Francis JH, Rampal R, Lacouture M, Brody LA, Ozkaya N, Dogan A, Rosen N, Iasonos A, Abdel-Wahab O, Hyman DM. Efficacy of MEK inhibition in patients with histiocytic neoplasms. Nature. 2019 Mar;567(7749):521-524. Epub 2019 Mar 13. link to original article contains dosing details in manuscript link to PMC article PubMed