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Histiocytosis in Children


The histiocytoses are a heterogeneous group of diseases which are characterized by the accumulation of reactive or neoplastic histiocytes in various tissues. They may be separated into three classes. Histiocytoses in which the predominant histiocytic cell type is of Langerhans cell phenotype are called Langerhans cell histiocytoses. They include Hand-Schüller-Christian syndrome, Letterer-Siwe disease and eosinophilic granuloma. In class II histiocytoses, mononuclear phagocytes other than Langerhans cells accumulate in various tissues and may cause tissue damage. Juvenile xanthogranuloma and xanthoma disseminatum belong to this group. Class III histiocytoses include all the malignant histiocytic diseases.




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