Abstract
In the context of histiocytosis syndromes, histiocytes are defined as a group of immune cells, including macrophages and dendritic cells. The term histiocytosis identifies a group of disorders that have in common the proliferation of cells of the mononuclear phagocyte and the dendritic cell systems. Macrophages have several key functions, including tissue remodeling, tumor repressing and promoting activities and in antigen-processing and immune stimulation; dendritic cells function primarily as antigen-presenting cells and immune system regulators. Although the principal pathological cells of these syndromes are histiocytes, the term histiocytosis is a very selective term in the sense that it does not include storage diseases, hyperlipidemic xanthomatosis, or granulomatous reaction in chronic infections, such as tuberculosis or foreign body granuloma. This chapter shows the ontogeny of histiocytes, lists the immunophenotypic markers of non-neoplastic macrophages and dendritic cells of the histiocyte system, and highlightes classification of histiocytic disorders.
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