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Overview of Histiocytic Disorders

By Jeffrey M. Lipton, MD, PhD, Professor of Pediatrics and Molecular Medicine; Professor; Chief, Hematology/Oncology and Stem Cell Transplantation, Hofstra Northwell School of Medicine, Hempstead, NY; The Center for Autoimmune and Musculoskeletal Disease, Feinstein Institute for Medical Research; Cohen Children’s Medical Center of New York
Carolyn Fein Levy, MD, Assistant Professor, Hofstra Northwell School of Medicine

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The histiocytic disorders are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes—either

  • Monocyte-macrophages (antigen-processing cells)

  • Dendritic cells (antigen-presenting cells)

Classifying histiocytic disorders is difficult (see Table: Histiocytic Disorders) and has changed over time as an understanding of the biology of these cells has evolved. There are other rare histiocytic disorders such as Erdheim-Chester disease, juvenile xanthogranuloma, and others.

Histiocytic Disorders


Usual Disorders*

Dendritic cell–related

Juvenile xanthogranuloma

Erdheim Chester disease


*Other, rare disorders exist in each category.

Includes the disorders formerly called eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schüller-Christian disease

Also called sinus histiocytosis with massive lymphadenopathy

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