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

• Published in: Blood Vol. 127; no. 22; pp. 2672 - 2681
• Main Authors: Emile, Jean-François, Abla, Oussama, Fraitag, Sylvie, Horne, Annacarin, Haroche, Julien, Donadieu, Jean, Requena-Caballero, Luis, Jordan, Michael B., Abdel-Wahab, Omar, Allen, Carl E., Charlotte, Frédéric, Diamond, Eli L., Egeler, R.Maarten, Fischer, Alain, Herrera, Juana Gil, Henter, Jan-Inge, Janku, Filip, Merad, Miriam, Picarsic, Jennifer, Rodriguez-Galindo, Carlos, Rollins, Barret J., Tazi, Abdellatif, Vassallo, Robert, Weiss, Lawrence M.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 02.06.2016; American Society of Hematology
• Subjects: 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; Review
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2016-01-690636

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.