Unknown

Dataset Information

0

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.

SUBMITTER: Emile JF 

PROVIDER: S-EPMC5161007 | biostudies-literature | 2016 Jun

REPOSITORIES: biostudies-literature

altmetric image

Publications


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.  ...[more]

Similar Datasets

| S-EPMC10133850 | biostudies-literature
| S-EPMC5262539 | biostudies-literature
| S-EPMC4662485 | biostudies-literature
| S-EPMC5716762 | biostudies-literature
| S-EPMC3483872 | biostudies-literature
| S-EPMC9252913 | biostudies-literature
| S-EPMC5480456 | biostudies-literature
| S-EPMC8742823 | biostudies-literature
| S-EPMC3179938 | biostudies-literature
| S-EPMC8702826 | biostudies-literature