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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 Table 1: Histiocytic Syndromes: Some Histiocytic Syndromes ) and has changed over time as an understanding of the biology of these cells has evolved.
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Table 1
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| Some Histiocytic Syndromes |
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Category
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Usual Disorders*
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Examples or Description
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Disorders of varied biologic behavior
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Dendritic cell–related
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Langerhans' cell histiocytosis
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Includes the disorders formerly called eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schüller-Christian disease
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Macrophage-related
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Primary hemophagocytic syndromes
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Familial
Sporadic
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Secondary hemophagocytic syndromes
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Infection
Cancer
Autoimmune disorders
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Rosai-Dorfman disease
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Also called sinus histiocytosis with massive lymphadenopathy
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Malignant disorders
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Leukemias
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Acute monocytic and myelomonocytic leukemia
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—
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Chronic myelomonocytic leukemia (CMML)
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Adult CMML
Childhood CMML (juvenile myelomonocytic leukemia)
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* 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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Last full review/revision November 2009 by Jeffrey M. Lipton, MD, PhD
Content last modified February 2012
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