Mixed histiocytic disorders: Nature versus nurture?

ABSTRACT

Histiocytic diseases arise from MAPK mutations in myeloid progenitors. Depending on whether the progenitor follows a dendritic cell or macrophage/monocyte lineage the final histology results in Langerhans cell histiocytosis, Rosai-Dorfman disease or Erdheim-Chester disease. Commentary on Friedman et al. Mixed histiocytic neoplasms A multicentre series revealing diverse somatic mutations and responses to targeted therapy. Br J Haematol 2024;205127-137.