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Overview of Histiocytic Syndromes

by Jeffrey M. Lipton, MD, PhD

The histiocytic syndromes are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes—either monocyte-macrophages (antigen-processing cells) or dendritic cells (antigen-presenting cells). Classifying these disorders is difficult (see Some Histiocytic Syndromes) 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.

Some Histiocytic Syndromes


Usual Disorders*

Examples or Description

Histiocytic disorders of varied biologic behavior

Dendritic cell–related

Langerhans cell histiocytosis

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


Primary hemophagocytic syndromes



Secondary hemophagocytic syndromes



Autoimmune disorders

Rosai-Dorfman disease

Also called sinus histiocytosis with massive lymphadenopathy

Malignant histiocytic disorders


Acute monocytic and myelomonocytic leukemia

Chronic myelomonocytic leukemia (CMML)

Adult CMML

Childhood CMML (juvenile myelomonocytic leukemia)

*Other, rare disorders exist in each category.

Adapted from Komp DM, Perry MC: Introduction: The histiocytic syndromes. Seminars in Oncology 18:1, 1991 and Favara BE, Feller AC, Pauli M, eds.: Contemporary classification of histiocytic disorders. Medical and Pediatric Oncology 29:157, 1997.

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