The histiocytic group of diseases includes disorders resulting from proliferations of cells of the monocyte-macrophage lineage, which encompass a diverse group of benign and potentially fatal diseases. The histiocytic syndromes are currently divided into two major categories: Langerhans cell histiocytoses and non-Langerhans cell histiocytoses. The histiocytic-appearing cells in the infiltrative lesions of Langerhans cell histiocytoses contain Birbeck or Langerhans granules and are believed to be related to the Langerhans cell. Birbeck or Langerhans granules are absent from the histiocytic cells of the non-Langerhans cell histiocytoses.
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