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, Lawrence M. Weiss, Society Histiocyte Society

Research output: Contribution to journalReview articlepeer-review

1290 Scopus citations

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.

Original languageEnglish
Pages (from-to)2672-2681
Number of pages10
JournalBlood
Volume127
Issue number22
DOIs
StatePublished - 2 Jun 2016

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