The histiocytic disorders are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes that are either
Dendritic cells (antigen-presenting cells)
Monocyte-macrophages (antigen-processing cells)
Classifying histiocytic disorders is difficult (see table Histiocytic Disorders Histiocytic Disorders ) and has changed over time as an understanding of the biology of these cells has evolved (1 General references The histiocytic disorders are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes that are either Dendritic cells (antigen-presenting cells) Monocyte-macrophages... read more ). Langerhans cell histiocytosis Langerhans Cell Histiocytosis Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. Most cases occur in children. Manifestations may include... read more
is the most common histiocytic disorder. There are other rare histiocytic disorders such as Erdheim-Chester disease and juvenile xanthogranuloma Juvenile xanthogranuloma Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. Most cases occur in children. Manifestations may include... read more
, some of which are now considered variants of Langerhans cell histiocytosis (2 General references The histiocytic disorders are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes that are either Dendritic cells (antigen-presenting cells) Monocyte-macrophages... read more ).
General references
1. 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
2. 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