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

Blood. 2016 Jun 2;127(22):2672-81. doi: 10.1182/blood-2016-01-690636. Epub 2016 Mar 10.


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 types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Dendritic Cells* / classification
  • Dendritic Cells* / pathology
  • Female
  • Histiocytic Disorders, Malignant* / classification
  • Histiocytic Disorders, Malignant* / pathology
  • Histiocytosis, Langerhans-Cell* / classification
  • Histiocytosis, Langerhans-Cell* / pathology
  • Histiocytosis, Non-Langerhans-Cell* / classification
  • Histiocytosis, Non-Langerhans-Cell* / pathology
  • Humans
  • Macrophages* / classification
  • Macrophages* / pathology
  • Male

Associated data

  • PIR/601859
  • PIR/602782