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

Jean François Emile, Oussama Abla, Sylvie Fraitag, Annacarin Horne, Julien Haroche, Jean Donadieu, Luis Requena-Caballero, Michael B. Jordan, Omar Abdel-Wahab, Carl E. Allen, Frédéric Charlotte, Eli L. Diamond, R. Maarten Egeler, Alain Fischer, Juana Gil Herrera, Jan Inge Henter, Filip Janku, Miriam Merad, Jennifer Picarsic, Carlos Rodriguez-GalindoBarret J. Rollins, Abdellatif Tazi, Robert Vassallo, Lawrence M. Weiss, Society Histiocyte Society

Research output: Contribution to journalReview article

371 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)2672-2681
Number of pages10
Issue number22
StatePublished - Jun 2 2016

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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    Emile, J. F., Abla, O., Fraitag, S., Horne, A., Haroche, J., Donadieu, J., Requena-Caballero, L., Jordan, M. B., Abdel-Wahab, O., Allen, C. E., Charlotte, F., Diamond, E. L., Egeler, R. M., Fischer, A., Herrera, J. G., Henter, J. I., Janku, F., Merad, M., Picarsic, J., ... Histiocyte Society, S. (2016). Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood, 127(22), 2672-2681.