What changed: Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms

When this page itself was last checked or edited.

FDA approval October 2022 for adults with ECD, RDD and LCH.

Vemurafenib (FDA approval 2017) or dabrafenib, often with a MEK inhibitor to reduce toxicity; long-term treatment at the lowest effective dose.

Diamond and colleagues, Nature Medicine 2019.

Observation for asymptomatic nodal disease; surgery for isolated masses; steroids, sirolimus, cladribine or MEK inhibitors for symptomatic or multifocal disease.

Erdheim-Chester disease with BRAF V600 mutation

First approval for any histiocytosis (VE-BASKET); November 2017.

L, C, R, M and H groups (Emile et al., Blood 2016).

Haroche and colleagues (Blood 2012) after Badalian-Very's LCH discovery (2010).

Destombes had reported cases in 1965.

A milestone in how this cancer is treated.