38                                                                                                                                                 Histiocytoses  ______________________________________________________________________________________

7.    Histiocytoses

           

7.1       General considerations

            The term “ histiocytoses” was coined for a group of diverse disorders with the common 

 characteristic of an increased number of histiocytes – a variety of different cell type primarily

including macrophages and dendritic cells.

 

A (re)classification system for histiocytoses was established by the Histiocyte Society.

Classification schema of histiocytoses
  • Dendritic cell or related disorders

            e.g. Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma

  • Macrophage or related disorders

           e.g. hemophagocytic lymphohistiocytosis, sinus histiocytosis with massive 

           lymphadenopathy (Rosai – Dorfman disease)

  • Malignant disorders

           e.g. monocytic leukemia (FAB M5, see indication no. 2), monocytic sarcoma, histiocytic

           sarcoma.

 

            Literature: for review

                        ARCECI, Eur. J. Cancer 35 (1999): 747 – 769 (incl. A commentary by

                        HENTER)