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Histiocytic disorders.

Kenneth L McClain1, Camille Bigenwald2, Matthew Collin3

  • 1Texas Children's Cancer Center, Department of Paediatrics, Baylor College of Medicine, Houston, TX, USA. klmcclai@txch.org.

Nature Reviews. Disease Primers
|October 8, 2021
PubMed
Summary
This summary is machine-generated.

Histiocytic disorders involve pathogenic myeloid cells. While sharing MAPK pathway mutations with cancers, diseases like Langerhans cell histiocytosis are not hyperproliferative, suggesting a distinct classification.

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • The term 'histiocytosis' historically unifies diseases with pathogenic myeloid cells resembling macrophages or dendritic cells.
  • Existing classifications link disease names to cell phenotypes, such as Langerhans cell histiocytosis (LCH) sharing CD207 antigen.

Purpose of the Study:

  • To clarify the classification and nomenclature of histiocytic disorders.
  • To differentiate non-hyperproliferative histiocytic disorders from malignant neoplasms.

Main Methods:

  • Review of current literature and classification schemes for histiocytic disorders.
  • Analysis of cellular origins, histological features, and molecular pathways (e.g., MAPK pathway mutations).

Main Results:

  • Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), juvenile xanthogranuloma (JXG), and Rosai-Dorfman disease (RDD) share activating MAPK pathway mutations.
  • These conditions, unlike malignant histiocytosis, are not hyperproliferative, prompting consideration of 'inflammatory myeloproliferative neoplasm' as a precise term.
  • Haemophagocytic lymphohistiocytosis is distinguished as a syndrome of immune dysregulation with extreme inflammation.

Conclusions:

  • Histiocytic disorders represent a spectrum of myeloid cell-driven conditions with distinct biological and clinical characteristics.
  • Accurate nomenclature is crucial for differentiating these entities from true malignancies and immune dysregulation syndromes.