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The Histiocyte Society reorganised more than a hundred histiocytic disorders into five groups, putting Langerhans cell histiocytosis and Erdheim-Chester disease together as clonal MAPK-driven neoplasms and Rosai-Dorfman disease in its own group.
Classification from the Histiocyte Society dividing histiocytic disorders into five groups: L (Langerhans cell histiocytosis, indeterminate cell histiocytosis, Erdheim-Chester disease and mixed forms), C (cutaneous and mucocutaneous non-Langerhans histiocytoses), R (Rosai-Dorfman disease and related), M (malignant histiocytoses) and H (haemophagocytic lymphohistiocytosis and macrophage activation syndrome).
It reflects the discovery that LCH and ECD are clonal neoplasms driven by MAPK pathway mutations, and its groupings were carried into the WHO 2022 classification.
The way OnCo groups the histiocytoses, and the recognition that LCH and ECD are cancers rather than inflammatory conditions, come from this classification.
Shares Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Blood.
Shares Rosai-Dorfman-Destombes disease, Blood.
Shares Erdheim-Chester disease, Blood.
Shares Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Blood.
Shares Rosai-Dorfman-Destombes disease, Erdheim-Chester disease.
Shares Rosai-Dorfman-Destombes disease, Erdheim-Chester disease.
Shares Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Erdheim-Chester disease.
Shares Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Erdheim-Chester disease.