Uncommon histiocytic disorders: the non-Langerhans cell histiocytoses

Pediatr Blood Cancer. 2005 Sep;45(3):256-64. doi: 10.1002/pbc.20246.


Background: Histiocytic disorders are currently identified by their component cells. The non-Langerhans Cell Histiocytoses (non-LCH) are a group of disorders defined by the accumulation of histiocytes that do not meet the phenotypic criteria for the diagnosis of Langerhans cells (LCs). The non-LCH consist of a long list of diverse disorders which have been difficult to categorize. A conceptual way to think of these disorders that make them less confusing and easier to remember is proposed based on immunophenotyping and clinical presentation.

Results: Clinically the non-LCH can be divided into 3 groups, those that predominantly affect skin, those that affect skin but have a major systemic component, and those that primarily involve extracutaneous sites, although skin may be involved. Immunohistochernically many of the non-LCH appear to arise from the same precursor cell namely the dermal dendrocyte. Juvenile Xanthogranuloma (JXG) is the model of the dermal dendrocyte-derived non-LCH. Other non-LCH with differing clinical presentation and occurring at different ages but with an identical immunophenotype appear to form a spectrum of the same disorder, deriving from the same precursor cell at different stages of maturation. They should be considered as members of a JXG family. Non-JXG family members include Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).

Conclusion: The non-LCH can be classified as JXG family and non-JXG family and subdivided according to fairly clear-cut clinical criteria. Utilization of this type of approach will allow better categorization, easier review of the literature and more accurate therapy decision-making.

Publication types

  • Review

MeSH terms

  • Child
  • Histiocytes / metabolism
  • Histiocytosis, Non-Langerhans-Cell* / classification
  • Histiocytosis, Non-Langerhans-Cell* / diagnosis
  • Histiocytosis, Non-Langerhans-Cell* / immunology
  • Histiocytosis, Non-Langerhans-Cell* / physiopathology
  • Humans
  • Models, Immunological
  • Xanthogranuloma, Juvenile / classification
  • Xanthogranuloma, Juvenile / diagnosis
  • Xanthogranuloma, Juvenile / immunology
  • Xanthogranuloma, Juvenile / physiopathology