title: Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages.
abstract: 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.
authors.affiliation: Research Unit EA4340, Versailles University, Paris-Saclay University, Boulogne, France; Pathology Department, Ambroise Paré Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Boulogne, France;
authors.forename: Jean-François
authors.identifier: 0000-0002-6073-4466
authors.initials: JF
authors.lastname: Emile
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada;
authors.forename: Oussama
authors.initials: O
authors.lastname: Abla
authors.affiliation: Pathology Department, Necker Hospital, Paris, France;
authors.forename: Sylvie
authors.initials: S
authors.lastname: Fraitag
authors.affiliation: Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden;
authors.forename: Annacarin
authors.initials: A
authors.lastname: Horne
authors.affiliation: Department of Internal Medicine and French Reference Center for Rare Auto-immune and Systemic Diseases, Institut E3M, AP-HP, Pitié-Salpêtrière Hospital, Paris, France; Université Pierre et Marie Curie University Paris 6, Paris, France;
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.affiliation: Research Unit EA4340, Versailles University, Paris-Saclay University, Boulogne, France; Pediatric Hematology, Trousseau Hospital, APHP, Paris, France;
authors.forename: Jean
authors.initials: J
authors.lastname: Donadieu
authors.affiliation: Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain;
authors.forename: Luis
authors.initials: L
authors.lastname: Requena-Caballero
authors.affiliation: Department of Pediatrics, Cincinnati Children's Hospital Medical Center and the University of Cincinnati College of Medicine, Cincinnati, OH;
authors.forename: Michael B
authors.initials: MB
authors.lastname: Jordan
authors.affiliation: Leukemia Service, Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center, New York, NY;
authors.forename: Omar
authors.initials: O
authors.lastname: Abdel-Wahab
authors.affiliation: Feigin Center, Texas Children's Cancer Center, Houston, TX;
authors.forename: Carl E
authors.initials: CE
authors.lastname: Allen
authors.affiliation: Université Pierre et Marie Curie University Paris 6, Paris, France; Pathology Department, Pitié-Salpétrière Hospital, Paris, France;
authors.forename: Frédéric
authors.initials: F
authors.lastname: Charlotte
authors.affiliation: Department of Neurology, Memorial Sloan Kettering Cancer Center, New York, NY;
authors.forename: Eli L
authors.initials: EL
authors.lastname: Diamond
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada;
authors.forename: R Maarten
authors.initials: RM
authors.lastname: Egeler
authors.affiliation: Necker Enfants Malades Hospital, AP-HP, Paris, France; Institut Imagine, Sorbonne Paris Cité, Université Paris Descartes, Paris, France;
authors.forename: Alain
authors.initials: A
authors.lastname: Fischer
authors.affiliation: Division of Clínical Immunology, Hospital General Universitario and Health Research Institute "Gregorio Marañón," Madrid, Spain;
authors.forename: Juana Gil
authors.initials: JG
authors.lastname: Herrera
authors.affiliation: Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden;
authors.forename: Jan-Inge
authors.initials: JI
authors.lastname: Henter
authors.affiliation: Department of Investigational Cancer Therapeutics (Phase I Clinical Trials Program), The University of Texas MD Anderson Cancer Center, Houston, TX;
authors.forename: Filip
authors.initials: F
authors.lastname: Janku
authors.affiliation: Mount Sinai School of Medicine, New York, NY;
authors.forename: Miriam
authors.initials: M
authors.lastname: Merad
authors.affiliation: Pathology Department, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, PA;
authors.forename: Jennifer
authors.initials: J
authors.lastname: Picarsic
authors.affiliation: Dana-Farber Cancer Institute, Boston, MA;
authors.forename: Carlos
authors.initials: C
authors.lastname: Rodriguez-Galindo
authors.affiliation: Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA; Department of Medicine, Brigham & Women's Hospital, Harvard Medical School, Boston, MA;
authors.forename: Barret J
authors.initials: BJ
authors.lastname: Rollins
authors.affiliation: Division of Pulmonary, Saint Louis Hospital, Paris, France;
authors.forename: Abdellatif
authors.initials: A
authors.lastname: Tazi
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, MN; and.
authors.forename: Robert
authors.initials: R
authors.lastname: Vassallo
authors.affiliation: Clarient Pathology Services, Aliso Viejo, CA.
authors.forename: Lawrence M
authors.initials: LM
authors.lastname: Weiss
mesh_terms: D000328:Adult; D003713:Dendritic Cells / Q000145:classification / Q000473:pathology; D005260:Female; D015620:Histiocytic Disorders, Malignant / Q000145:classification / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000145:classification / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000145:classification / Q000473:pathology; D006801:Humans; D008264:Macrophages / Q000145:classification / Q000473:pathology; D008297:Male