Nosology and Pathology of Langerhans Cell Histiocytosis

Abstract

The classification of the histiocytoses has evolved based on new understanding of the cell of origin as a bone marrow precursor. Although the pathologic features of the histiocytoses have not changed per se, molecular genetic information now needs to be integrated into the diagnosis. The basic lesions of the most common histiocytoses, their patterns in different sites, and ancillary diagnostics are now just one part of the classification. As more is understood about the cell of origin and molecular biology of the histiocytoses, future classifications will be refined.

Keywords: Congenital and acquired disorders; Erdheim-Chester disease; Juvenile xanthogranuloma; Langerhans cell histocytosis; Rosai-Dorfman disease.