Classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages

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Classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages is an evolving classification published by the Histiocyte Society.

The version published in 2016 consists of five groups:[1]

Group Diseases Details
L group Langerhans cell histiocytosis (LCH), indeterminate cell histiocytosis, Erdheim-Chester disease (ECD), mixed LCH/ECD
C group cutaneous non-LCH, cutaneous non-LCH with major systemic component cutaneous non-LCH subdivided into: XG family JXG, non-XG family
R group familial Rosai-Dorfman disease (RDD), sporadic RDD sporadic RCC includes: classical RDD, extra-nodal RDD, RDD with neoplasia or autoimmune disease, unclassified RDD
M group primary malignant histiocytoses, secondary malignant histiocytoses secondary malignant histiocytosis follow another hematologic malignancy; subclassified as: histiocytic, interdigitating, Langerhans, indeterminate cell
H group primary hemophagocytic lymphohistiocytosis (HLH), secondary hemophagocytic lymphohistiocytosis, HLH or unknown/uncertain origin secondary HLH is non-Mendelian

See also

References

  1. Emile, JF.; Abla, O.; Fraitag, S.; Horne, A.; Haroche, J.; Donadieu, J.; Requena-Caballero, L.; Jordan, MB. et al. (06 2016). "Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages.". Blood 127 (22): 2672-81. doi:10.1182/blood-2016-01-690636. PMID 26966089.