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Other names: Histiocytic disorders[1]
TypesLangerhans cell histiocytosis (LCH), juvenile xanthogranuloma, Erdheim-Chester disease, hemophagocytic syndrome, Rosai-Dorfman disease[1]

Histiocytosis is a group of disorders that result in excessive number of histiocytes (dendritic cells or monocyte macrophages).[1] Symptoms are variable.[2] Commonly affected organs include skin, bone, lungs, lymph nodes, nervous system, and heart.[2]

The underlying mechanism may involved genetic changes in the mitogen-activated protein (MAP) kinase cell-signaling pathway.[2] Diagnosis is based on tissue biopsy, symptoms, and medical imaging.[2] Types include Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma, Erdheim-Chester disease, hemophagocytic syndrome, and Rosai-Dorfman disease.[1]

Outcomes range from the condition resolving without treatment to fatal disease.[2]

Histiocytosis is rare.[2] LCH affects about 7 per million people under the age of 15 year year; though may occur at any age.[2] Occasionally and confusingly, the term "histiocytosis" is sometimes used to refer to individual diseases.


Types of LCH have also been known as "eosinophilic granuloma", "Hand-Schuller-Christian disease", "Letterer-Siwe disease", and "histiocytosis X". (See the article Langerhans cell histiocytosis for details).

Alternatively, histiocytoses may be divided into the following groups:[3]: 714–724 

Lymphohistiocytosis is "a widespread infiltrate of non-malignant lymphocytes and macrophages, involving principally the liver, spleen and central nervous system and associated with a severe lymphoid atrophy."[4]


Sinus histiocytosis, a common feature in lymph node biopsies, is characterized by dilated sinuses containing variable numbers of histiocytes.[5]


There are competing systems of classification. In the 1999 system proposed by the World Health Organization, they are divided into three categories.[6][7] However, the classifications in ICD10 and MeSH are slightly different.

Langerhans cell histiocytosis (LCH) I D76.0 Langerhans-cell histiocytosis
Juvenile xanthogranuloma (JXG) II D76.3 non-Langerhans-cell histiocytosis
Hemophagocytic lymphohistiocytosis (HLH) II D76.1 non-Langerhans-cell histiocytosis
Niemann–Pick disease II E75.2 non-Langerhans-cell histiocytosis
Sea-blue histiocytosis II - non-Langerhans-cell histiocytosis
Acute monocytic leukemia III C93.0 malignant histiocytic disorders
Malignant histiocytosis III C96.1 malignant histiocytic disorders
Erdheim–Chester disease II C96.1 malignant histiocytic disorders



Patients and families can gain support and educational materials from the Histiocytosis Association Archived 2021-04-16 at the Wayback Machine, or the Histiocytosis Research Trust Archived 2019-01-07 at the Wayback Machine. Information concerning histiocytosis and clinicians located in European countries may be found in many languages at the web portal of Euro Histio Net Archived 2010-12-29 at the Wayback Machine (EHN). This is a project funded by the European Union, coordinated by Jean Donadieu, APHP Archived 2011-10-15 at the Wayback Machine, Paris, France. Additional information about Erdheim–Chester disease can be obtained from the ECD Global Alliance Archived 2021-06-09 at the Wayback Machine.

The Histiocyte Society, a nonprofit organization, is a group of more than 200 physicians and scientists from around the world committed to improving the lives of patients with histiocytic disorders by conducting clinical and laboratory research into the causes and treatment of this disease. The Society has instituted several clinical trials and treatment plans.[8][9]

The North American Consortium for Histiocytosis (NACHO) Archived 2021-08-15 at the Wayback Machine is a group of institutions that collaborate on scientific and clinical research for histiocytic diseases. Established in 2014 by 12 institutions, it was funded through a consortium grant from the St. Baldrick's Foundation.

Other animals

Other animals may also be affected.


  1. 1.0 1.1 1.2 1.3 "Overview of Histiocytic Disorders - Hematology and Oncology". Merck Manuals Professional Edition. Archived from the original on 2 June 2022. Retrieved 22 October 2022.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Emile, Jean-François; Cohen-Aubart, Fleur; Collin, Matthew; Fraitag, Sylvie; Idbaih, Ahmed; Abdel-Wahab, Omar; Rollins, Barrett J; Donadieu, Jean; Haroche, Julien (July 2021). "Histiocytosis". The Lancet. 398 (10295): 157–170. doi:10.1016/S0140-6736(21)00311-1.
  3. James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
  4. Goldberg, J; Nezelof, C (1986), "Lymphohistiocytosis: a multi-factorial syndrome of macrophagic activation clinico-pathological study of 38 cases", Hematol Oncol, 4 (4): 275–289, PMID 3557322.
  5. Egan, Caoimhe; Jaffe, Elaine S. (2018). "Non-neoplastic histiocytic and dendritic cell disorders in lymph nodes". Seminars in Diagnostic Pathology. 35 (1): 20–33. doi:10.1053/j.semdp.2017.11.002. ISSN 0740-2570. PMC 5803315.
  6. Harris N, Jaffe E, Diebold J, Flandrin G, Muller-Hermelink H, Vardiman J, Lister T, Bloomfield C (1999). "The World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues. Report of the Clinical Advisory Committee meeting, Airlie House, Virginia, November, 1997". Ann Oncol. 10 (12): 1419–32. doi:10.1023/A:1008375931236. PMID 10643532.
  7. Histiocytosis at eMedicine
  8. Tebbi, Cameron K. (16 September 2020). Kanwar, Vikramjit S (ed.). "What is the Histiocyte Society classification of histiocytosis syndromes?". Medscape. Archived from the original on 20 January 2021. Retrieved 4 December 2020.
  9. Chang, Karen L.; Snyder, David S. (2007). "17. Langerhans Cell Histiocytosis". In Stephen M. Ansell (ed.). Rare Hematological Malignancies. Springer Science & Business Media. p. 383. ISBN 978-0-387-73743-0. Archived from the original on 2021-08-29. Retrieved 2021-08-15.

External links

External resources