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'''''Synonyms and related keywords:''''' Hand-Schuller-Christian disease, Histiocytosis X, Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis and Letterer-Siwe disease
'''''Synonyms and related keywords:''''' Hand-Schuller-Christian disease, Histiocytosis X, Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis and Letterer-Siwe disease


==Overview==
==[[Histiocytosis overview|Overview]]==
In [[medicine]], '''histiocytosis''' is an excessive number of [[histiocyte]]s,that is an excessive number of ''tissue [[macrophages]]'', and is typically used to refer to a group of rare diseases which share this as a characteristic. Occasionally and confusingly it is sometimes used to refer to individual diseases.
==[[Histiocytosis classification|Classification]]==
==[[Histiocytosis pathophysiology |Pathophysiology]]==
==[[Histiocytosis causes|Causes]]==
==[[Histiocytosis differential diagnosis|Differentiating Histiocytosis from other Diseases]]==
==[[Histiocytosis epidemiology and demographics|Epidemiology and Demographics]]==
==[[Histiocytosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
==Diagnosis==
[[Histiocytosis history and symptoms|History and Symptoms]] | [[Histiocytosis physical examination|Physical Examination]] | [[Histiocytosis laboratory findings|Laboratory Findings]] | [[Histiocytosis x ray|X Ray]] | [[Histiocytosis CT|CT]] | [[Histiocytosis MRI|MRI]] | [[Histiocytosis other imaging findings|Other Imaging Findings]] | [[Histiocytosis other diagnostic studies|Other Diagnostic Studies]]


According to the Histiocytosis Association of America, 1 in 200,000 children in the United States are born with histiocytosis each year.<ref>[http://www.histio.org/site/c.kiKTL4PQLvF/b.1810505/k.F16D/Disease_Information.htm Disease information] at the Histiocytosis Association of America</ref> HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10. The University of California, San Francisco, states that the disease usually occurs from birth to age 15.<ref>http://www.ucsfhealth.org/childrens/medical_services/cancer/histio/conditions/histio/signs.html</ref>
==Treatment==
 
[[Histiocytosis medical therapy|Medical Therapy]] | [[Histiocytosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Histiocytosis future or investigational therapies|Future or Investigational Therapies]]
Histiocytosis (and [[malignant histiocytosis]]) are both important in veterinary as well as human pathology.
==Case Studies==
 
:[[Histiocytosis case study one|Case #1]]
==Presentation==
 
Histiocytosis is frequently associated with [[diabetes insipidus]], even after several years of diagnosis and successful therapy.
 
==Classification, and relationships to other conditions==
 
There are competing systems for classifying histiocytoses. According to the 1999 classification proposed by the [[World Health Organization]], they can be divided into three categories.<ref>{{cite journal |author=Harris N, Jaffe E, Diebold J, Flandrin G, Muller-Hermelink H, Vardiman J, Lister T, Bloomfield C |title=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 |journal=Ann Oncol |volume=10 |issue=12 |pages=1419-32 |year=1999 |pmid=10643532}}</ref>. However, the classifications in ICD10 and MeSH are slightly different, as shown below:
 
{| class="wikitable"
| '''Name''' || '''WHO''' || '''[[ICD10]]''' ||  '''[[MeSH]]'''
|-  
| [[Langerhans cell histiocytosis]] (LCH) || I || {{ICD10|D|76|0|d|70}}  || Langerhans-cell histiocytosis
|-
| [[Juvenile xanthogranuloma]] (JXG) || II || {{ICD10|D|76|3|d|70}}  ||  non-Langerhans-cell histiocytosis
|-
| [[Hemophagocytic lymphohistiocytosis]] (HLH)  || II  || {{ICD10|D|76|1|d|70}} ||  non-Langerhans-cell histiocytosis
|-
| [[Niemann-Pick disease]] || - || {{ICD10|E|75|2|e|70}} || non-Langerhans-cell histiocytosis
|-
| [[Sea-blue histiocyte syndrome]] || - || - || non-Langerhans-cell histiocytosis
|-
| [[Acute monocytic leukemia]] || III    || {{ICD10|C|93|0|c|81}} || malignant histiocytic disorders
|-
| [[Malignant histiocytosis]] || III || {{ICD10|C|96|1|c|81}} || malignant histiocytic disorders
|-
| [[Erdheim-Chester disease]] || - || {{ICD10|C|96|1|c|81}} || malignant histiocytic disorders
|}
 
Types of LCH have also been known as "[[Eosinophilic Granuloma]]", "[[Hand-Schuller-Christian Disease]]", "[[Letterer-Siwe Disease]]", and "[[Histiocytosis X]]". (See [[Histiocytosis%2C_langerhans-cell#History|LCH history]] for details).
 
==Common treatments==
*Chemotherapy
**[[Cladribine]] or [[2CDA]] or [[leustatin]]
**[[Etoposide]]
**[[Vinblastine]] or [[Velban]]
 
==Organizations==
Patients and families can gain some support by contacting patient organisations such as the Histiocytosis Association of America ([http://www.histio.org]) or the Histiocytosis Research Trust ([http://www.hrtrust.org]). The [http://www.histio.org Histiocytosis Association of America] has several stable and proven ''treatment protocols'' available only for physicians ([http://histio.org/society/inforequest.shtml]).
 
==References==
{{reflist|2}}


{{Hematology}}
{{Hematology}}

Revision as of 14:39, 21 September 2012

Histiocytosis
ICD-10 C96.1, D76.0
ICD-9 202.3, 277.89
MeSH D015614

Histiocytosis Microchapters

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Classification

Pathophysiology

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Epidemiology and Demographics

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Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

X Ray

CT

MRI

Other Imaging Findings

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Future or Investigational Therapies

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Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and related keywords: Hand-Schuller-Christian disease, Histiocytosis X, Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis and Letterer-Siwe disease

Overview

Classification

Pathophysiology

Causes

Differentiating Histiocytosis from other Diseases

Epidemiology and Demographics

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | X Ray | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

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