How are histiocytosis disorders classified?

Updated: Sep 16, 2020
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Answer

Classification of diseases involving histiocytic and dendritic cells is difficult because it requires inclusion of a broad range of diverse diseases. Therefore, most classifications are incomplete. The location of lesions and the extent of the disease substantially affect the course of the disease and the patient's prognosis. Thus, decisions regarding treatment are usually based on the extent of the disease and evidence of critical organ (risk organ) dysfunction (ie, lung, liver, spleen, bone marrow).

Since the initial classification of histiocytosis disorders in 1987 by the Histiocyte Society, this organization has made a number of revisions this categorization. It should be noted that several other classifications by other groups or sources are also available, some of which are shown below. The Histiocyte Society's 2016 revision of the classification of histiocytosis and neoplasms of the macrophage and dendritic cell lineages is as follows. [60]  


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