Sheila Weitzman is a Senior Staff Oncologist and Professor of Pediatrics; Ronald Jaffe is a Professor of Pathology.
Uncommon histiocytic disorders: The non-Langerhans cell histiocytoses†
Article first published online: 16 NOV 2004
Copyright © 2004 Wiley-Liss, Inc.
Pediatric Blood & Cancer
Volume 45, Issue 3, pages 256–264, September 2005
How to Cite
Weitzman, S. and Jaffe, R. (2005), Uncommon histiocytic disorders: The non-Langerhans cell histiocytoses. Pediatr. Blood Cancer, 45: 256–264. doi: 10.1002/pbc.20246
- Issue published online: 13 JUL 2005
- Article first published online: 16 NOV 2004
- Manuscript Accepted: 21 SEP 2004
- Manuscript Received: 15 JUN 2004
- histiocytic disorders;
- Langerhans cell histiocytosis;
- non-Langerhans cell histiocytosis
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.
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).
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. © 2004 Wiley-Liss, Inc.