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Overview of Histiocytic Disorders

By

Jeffrey M. Lipton

, MD, PhD, Zucker School of Medicine at Hofstra/Northwell;


Carolyn Fein Levy

, MD, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell

Last full review/revision Dec 2021
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Topic Resources

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

  • Dendritic cells (antigen-presenting cells)

  • Monocyte-macrophages (antigen-processing cells)

Classifying histiocytic disorders is difficult (see table Histiocytic Disorders Histiocytic Disorders The histiocytic disorders are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes that are either Dendritic cells (antigen-presenting cells) Monocyte-macrophages... read more ) and has changed over time as an understanding of the biology of these cells has evolved (1 General references The histiocytic disorders are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes that are either Dendritic cells (antigen-presenting cells) Monocyte-macrophages... read more ). Langerhans cell histiocytosis Langerhans Cell Histiocytosis Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. Most cases occur in children. Manifestations may include... read more Langerhans Cell Histiocytosis is the most common histiocytic disorder. There are other rare histiocytic disorders such as Erdheim-Chester disease and juvenile xanthogranuloma Juvenile xanthogranuloma Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. Most cases occur in children. Manifestations may include... read more Juvenile xanthogranuloma , some of which are now considered variants of Langerhans cell histiocytosis (2 General references The histiocytic disorders are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes that are either Dendritic cells (antigen-presenting cells) Monocyte-macrophages... read more ).

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General references

  • 1. Emile JF, Abla O, Fraitag S, et al: Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 127(22):2672–2681, 2016. doi: 10.1182/blood-2016-01-690636

  • 2. Höck, M., Zelger, B., Schweigmann, G. et al: The various clinical spectra of juvenile xanthogranuloma: Imaging for two case reports and review of the literature. BMC Pediatr 19, 128 ,2019. doi: 10.1186/s12887-019-1490-y

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Langerhans Cell Histiocytosis
A 10-year-old girl is brought to the clinic by her father because she has had pain in her right upper leg for the past 3 months. On physical examination, the patient is unable to bear weight on the limb. Edema of the upper leg and tenderness to palpation are noted. X-rays show 2 bone lesions with sharp margins and a punched-out appearance. Which of the following is the most appropriate definitive step in diagnosis?
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