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Overview of Histiocytic Disorders


Jeffrey M. Lipton

, MD, PhD, Hofstra Northwell School of Medicine, Hempstead, NY;

Carolyn Fein Levy

, MD, Hofstra Northwell School of Medicine

Last full review/revision Jul 2019| Content last modified Jul 2019
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The histiocytic disorders are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes that are 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. Langerhans cell histiocytosis is the most common histiocytic disorder. There are other rare histiocytic disorders such as Erdheim-Chester disease and juvenile xanthogranuloma, some of which are now considered variants of Langerhans cell histiocytosis.


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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