Article
Rosai-Dorfman-Destombes disease - histiocytic disorder with inflammatory manifestation.
Klinicka onkologie : casopis Ceske a Slovenske onkologicke spolecnosti - 1 Jan 2022
Adam Z, Adamová Z, Pour L, Řehák Z, Koukalová R, Král Z
Abstract excerpt
BACKGROUND: Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytosis characterized by accumulation of activated histiocytes within affected tissues. Although the immunophenotype of this disease was described, the pathophysiology of this disease is still not sufficiently understood. Recent studies have found NRAS, KRAS, MAP2K1, and ARAF mutations in RDD lesions, raising the possibility of a clonal origin in...
Topics
- Histiocytes
- Histiocytosis, Sinus
- Humans
- Mutation
