Histiocytic syndromes

Monocytes are formed in the marrow and move through the peripheral blood into the tissues where they become histiocytes, either in the mononuclear phagocytic system (MPS) or the dendritic cell system (DCS). MPS cells are antigen processing, are predominantly phagocytic and include many organ-specific cells such as Kupffer cells and pulmonary alveolar macrophages. DCS cells include tissue-based Langerhans cells (LC) which are antigen presenting. There is a variety of syndromes where histiocytes proliferate and malfunction and some of these carry a high mortality. A few are clonal neoplasms but most are produced by cytokine disturbances. In 1991 a new classification of histiocytic syndromes was set out as shown:

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