Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages Review uri icon


MeSH Major

  • Dendritic Cells
  • Histiocytic Disorders, Malignant
  • Histiocytosis, Langerhans-Cell
  • Histiocytosis, Non-Langerhans-Cell
  • Macrophages


  • The histiocytoses are rare disorders characterized by the accumulation of macrophage, dendritic cell, or monocyte-derived cells in various tissues and organs of children and adults. More than 100 different subtypes have been described, with a wide range of clinical manifestations, presentations, and histologies. Since the first classification in 1987, a number of new findings regarding the cellular origins, molecular pathology, and clinical features of histiocytic disorders have been identified. We propose herein a revision of the classification of histiocytoses based on histology, phenotype, molecular alterations, and clinical and imaging characteristics. This revised classification system consists of 5 groups of diseases: (1) Langerhans-related, (2) cutaneous and mucocutaneous, and (3) malignant histiocytoses as well as (4) Rosai-Dorfman disease and (5) hemophagocytic lymphohistiocytosis and macrophage activation syndrome. Herein, we provide guidelines and recommendations for diagnoses of these disorders.

publication date

  • June 2, 2016



  • Review



  • eng

PubMed Central ID

  • PMC5161007

Digital Object Identifier (DOI)

  • 10.1182/blood-2016-01-690636

PubMed ID

  • 26966089

Additional Document Info

start page

  • 2672

end page

  • 81


  • 127


  • 22