What is the WHO classification of histiocytic disorders?

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

The classification of histiocytic disorders the World Health Organization (WHO) has proposed is as follows: [61]

  • Class I - Langerhans cell histiocytosis

  • Class II

    • Histiocytosis of mononuclear phagocytes other than Langerhans cells

    • Familial and reactive hemophagocytic lymphohistiocytosis (HLH)

    • Sinus histiocytosis with massive lymphadenopathy (SHML), Rosai-Dorfman disease

    • Juvenile xanthogranuloma (JXG)

    • Reticulohistiocytoma

  • Class III

    • Malignant histiocytic disorders

    • Acute monocytic leukemia (FAB M5)

    • Malignant histiocytosis

    • True histiocytic lymphoma

The WHO classification of neoplastic disorders of histiocytes and dendritic cells is as follows:

  • Macrophage or histiocyte related

    • Histiocytic sarcoma, mainly localized

    • Generalized malignant histiocytosis (may be related to acute monocytic leukemia)

  • Dendritic-cell related

    • 2A - Localized or generalized Langerhans cell histiocytosis

    • 2B - Langerhans cell sarcoma

    • 2C - Interdigitating dendritic cell sarcoma

2D - Follicular dendritic cell sarcoma or tumor 


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