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

2016/03/10 by Jean‐François Emile, Jean-François Emile, Oussama Abla +29 · 31 citations
Medicine · #Histiocytic Disorders and Treatments #Parvovirus B19 Infection Studies #Viral-associated cancers and disorders

paper · pdf · doi:10.1182/blood-2016-01-690636

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.

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