Histiocytic Disorders

Category

Usual Disorders*

Dendritic cell–related

Langerhans cell histiocytosis (LCH)†

Juvenile xanthogranuloma (JXG)

Erdheim-Chester disease (ECD)

Macrophage-related

Primary hemophagocytic syndromes

Secondary hemophagocytic syndromes

Rosai-Dorfman disease (RDD)‡

* Other, rare disorders exist in each category.

† Includes the disorders formerly called eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schüller-Christian disease

‡ Also called sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman-Destombes disease

See also Emile JF, Abla O, Fraitag S, et al: Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 127(22):2672–2681, 2016. doi: 10.1182/blood-2016-01-690636 and Höck, M., Zelger, B., Schweigmann, G. et al: The various clinical spectra of juvenile xanthogranuloma: Imaging for two case reports and review of the literature. BMC Pediatr 19, 128, 2019. doi: 10.1186/s12887-019-1490-y