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-Galindo, Barret J. Rollins, Abdellatif Tazi, Robert Vassallo and Lawrence M. Weiss


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 one hundred 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 muco-cutaneous, and (3) malignant histiocytoses as well as (4) Rosai Dorfman Disease and (5) haemophagocytic lymphohistiocytosis and macrophage activation syndrome. Herein we provide guidelines and recommendations for diagnoses of these disorders.

  • Submitted January 12, 2016.
  • Accepted March 2, 2016.