LCH
MCID: LNG024
MIFTS: 56

Langerhans-Cell Histiocytosis (LCH) malady

Rare diseases, Respiratory diseases, Bone diseases, Endocrine diseases, Blood diseases, Cancer diseases, Immune diseases categories
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Summaries for Langerhans-Cell Histiocytosis

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Sources:
8Disease Ontology, 43NIH Rare Diseases, 65Wikipedia, 47OMIM, 33MalaCards
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NIH Rare Diseases:43 Langerhans cell histiocytosis (lch) is a disorder that primarily affects children, but is also found in adults of all ages. people with lch produce too many langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. in people with lch, these cells multiply excessively and build up in certain areas of the body, causing tumors called granulomas to form. the symptoms vary among affected individuals, and the cause of lch is unknown. in most cases, this condition is not life-threatening. some people do experience life-long problems associated with lch. last updated: 10/18/2013

MalaCards: Langerhans-Cell Histiocytosis, also known as langerhans cell histiocytosis, is related to histiocytosis and sarcoma, and has symptoms including diabetes insipidus, premature eruption of teeth/natal teeth and tapered fingers. An important gene associated with Langerhans-Cell Histiocytosis is CD207 (CD207 molecule, langerin). The drug thalidomide has been mentioned in the context of this disorder. Affiliated tissues include lymph node, bone and spleen.

Disease Ontology:8 A histiocytosis that is characterized by clonal proliferation of langerhans cells.

Wikipedia:65 Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of Langerhans... more...

Description from OMIM:47 604856,246400

Aliases & Classifications for Langerhans-Cell Histiocytosis

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8Disease Ontology, 43NIH Rare Diseases, 21Genetics Home Reference, 10DISEASES, 45Novoseek, 49Orphanet, 47OMIM, 62UMLS, 58SNOMED-CT, 40NCIt, 27ICD9CM, 35MeSH, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

49
langerhans cell histiocytosis:
Prevalence: 1-9/100000; Age of onset: Variable; Age of death: Normal
letterer-siwe disease:
Prevalence: 1-9/1000000; Age of onset: Neonatal/infancy


Aliases & Descriptions:

langerhans-cell histiocytosis 8 10
langerhans cell histiocytosis 43 21 45 49 47
letterer-siwe disease 8 43 10 49 47
histiocytosis x 8 43 21 45 49
eosinophilic granuloma 43 45 49 62
langerhans cell granulomatosis 8 21 49
lch 43 21
letterer-siwe disease involving lymph nodes of inguinal region and lower limb 8
letterer-siwe disease of lymph nodes of inguinal region and/or lower limb 8
letterer-siwe disease of lymph nodes of inguinal region amd/or lower limb 8
letterer-siwe disease of lymph nodes of inguinal region and lower limb 8
letterer-siwe disease involving lymph nodes of axilla and upper limb 8
letterer-siwe disease involving lymph nodes of head, face, and neck 8
letterer-siwe disease involving lymph nodes of head, face and neck 8
letterer-siwe disease of lymph nodes of axilla and/or upper limb 8
letterer-siwe disease of lymph nodes of head, face and/or neck 8
letterer-siwe disease of lymph nodes of axilla and upper limb 8
letterer-siwe disease involving lymph nodes of multiple sites 8
letterer-siwe disease of lymph nodes of head, face and neck 8
letterer-siwe disease involving intra-abdominal lymph nodes 8
letterer-siwe disease involving intrathoracic lymph nodes 8
letterer-siwe disease involving intrapelvic lymph nodes 8
letterer-siwe disease of lymph nodes of multiple sites 8
acute and disseminated langerhans cell histiocytosis 49
letterer-siwe disease of intra-abdominal lymph nodes 8
chronic and multifocal langerhans cell histiocytosis 49
chronic and localized langerhans cell histiocytosis 49
letterer-siwe disease of intrathoracic lymph nodes 8
letterer-siwe disease of intrapelvic lymph nodes 8
letterer-siwe disease involving spleen 8
multifocal eosinophilic granuloma 49
hand-schüller-christian syndrome 43
hand-schüller-christian disease 49
letterer-siwe disease of spleen 8
langerhan's cell histiocytosis 8
histiocytosis, langerhans-cell 62
hashimoto-pritzger disease 21


External Ids:

Disease Ontology8 DOID:2571
ICD9CM27 202.5
MESH via Orphanet36 D006646, D004803, C538636
ICD10 via Orphanet26 C96.0, C96.5, C96.6
SNOMED-CT via Orphanet59 128809007, 190955000, 65399007 129000002, 39795003, 118614007, 128812005, more
UMLS via Orphanet63 C0019621, C0014461, C0023381

Related Diseases for Langerhans-Cell Histiocytosis

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Langerhans-Cell Histiocytosis:



Diseases related to langerhans-cell histiocytosis

Symptoms for Langerhans-Cell Histiocytosis

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47OMIM, 49Orphanet
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Symptoms by clinical synopsis from OMIM:

246400

Clinical features from OMIM:

604856,246400

Symptoms:

49 (show all 31)
  • diabetes insipidus
  • premature eruption of teeth/natal teeth
  • tapered fingers
  • lymphadenopathy/polyadenopathies
  • bone pain
  • bone tumefaction/swelling
  • proptosis/exophthalmos
  • mutiple fractures/bone fragility
  • follicular/erythematous/edematous papules/milium
  • emphysema
  • hydrarthrosis/articular/joint effusion
  • fever/chilling
  • asthenia/fatigue/weakness
  • anomalies of teeth and dentition
  • chronic/relapsing otitis
  • hepatomegaly/liver enlargement (excluding storage disease)
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • hemiplegia/diplegia/hemiparesia/limb palsy
  • anomalies of tongue, gingiva and oral mucosa
  • thoracic/chest pain
  • cough
  • lymphoma
  • chronic obstructive pulmonary disease/copd/obstructive respiratory syndrome
  • facial pain/cephalalgia/migraine
  • wasted (excluding lipodystrophy)/poorly muscled build/cachexy
  • lung/bronchopulmonary neoplasm/tumor/carcinoma/cancer
  • osteolysis/osteoclasia/bone destruction/erosions
  • pulmonary hypertension
  • lung/pulmonary infiltrates
  • interstitial/restrictive pneumopathy/restrictive respiratory syndrome
  • hyperhidrosis/increased sweating

Drugs & Therapeutics for Langerhans-Cell Histiocytosis

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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

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Search NIH Clinical Center for Langerhans-Cell Histiocytosis

Inferred drug relations via UMLS62/NDF-RT41:

Genetic Tests for Langerhans-Cell Histiocytosis

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Anatomical Context for Langerhans-Cell Histiocytosis

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33MalaCards
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MalaCards organs/tissues related to Langerhans-Cell Histiocytosis:

33
Lymph node, Bone, Spleen, Skin, Lung, Bone marrow, Liver, Thyroid, Pituitary, Myeloid, Tongue, T cells, Colon, Testes, Breast, Spinal cord, Thymus, B cells, Heart, Monocytes

Animal Models for Langerhans-Cell Histiocytosis or affiliated genes

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Publications for Langerhans-Cell Histiocytosis

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52PubMed
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Articles related to Langerhans-Cell Histiocytosis:

(show top 50)    (show all 638)
idTitleAuthorsYear
1
Pulmonary Langerhans cell histiocytosis in children: A spectrum of radiologic findings. (23769766)
2014
2
Skeletal involvement in Langerhans cell histiocytosis. (23907805)
2013
3
Rapid resolution of pulmonary Langerhans cell histiocytosis. (23749831)
2013
4
Differentiating pulmonary lymphangioleiomyomatosis from pulmonary langerhans cell histiocytosis and Birt-Hogg-Dube syndrome. (24339506)
2013
5
Pulmonary adenocarcinoma in a young patient of pulmonary langerhans cell histiocytosis (PLCH). (23857403)
2013
6
Effects of treatment with a combination of cardiac rehabilitation and bosentan in patients with pulmonary Langerhans cell histiocytosis associated with pulmonary hypertension. (23897898)
2013
7
Multisystem Langerhans cell histiocytosis in adult. (22470214)
2012
8
Juvenile xanthogranuloma developing after treatment of Langerhans cell histiocytosis: case report and literature review. (22977671)
2012
9
Langerhans' cell histiocytosis of the temporal bone. (21725272)
2012
10
Langerhans cell histiocytosis of the temporal bone. (21970849)
2012
11
Adult Langerhans cell histiocytosis in a middle aged Ethiopian woman. (22924288)
2012
12
Pulmonary langerhans cell histiocytosis. (22429393)
2012
13
Langerhans cell histiocytosis: two clinical presentations in the same patient. (22301045)
2012
14
Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy. (22835048)
2012
15
Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: a case of extramedullary hematopoiesis. (21922646)
2012
16
A case of coexisting Warthin tumor and langerhans cell histiocytosis associated with necrosis, eosinophilic abscesses and a granulomatous reaction in intraparotid lymph nodes. (21769315)
2011
17
Treatment of patients with hypothalamic-pituitary lesions as adult-onset Langerhans cell histiocytosis. (22015494)
2011
18
Langerhans cell histiocytosis preceding the development of juvenile xanthogranuloma: a case and review of recent developments. (21793710)
2011
19
Localized Langerhans cell histiocytosis of the stomach treated by endoscopic submucosal dissection. (21837608)
2011
20
Clinical pathologic conference case 2: Langerhans cell histiocytosis. (21874507)
2011
21
Case report: Langerhans cell histiocytosis presenting as massive cervical adenopathy and retropharyngeal thickening: importance of chest radiography and coordinated airway and anesthetic management. (20145506)
2010
22
Uncommon features of pulmonary Langerhans' cell histiocytosis: analysis of 11 cases and a review of the literature. (20193493)
2010
23
Cutaneous Langerhans cell histiocytosis in an elderly woman. (21062600)
2010
24
A role for ITPA variants in the clinical course of pulmonary Langerhans' cell histiocytosis? (20930204)
2010
25
Langerhans cell histiocytosis with thyroid and lung involvement in a child: a case report. (20224443)
2010
26
Langerhans cell histiocytosis in an adult: good response of cutaneous lesions to acitretin. (20184614)
2010
27
MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis: experience on seven patients. (19861578)
2010
28
Multifocal Langerhans cell histiocytosis with infiltrative pelvic lesions: PET/CT imaging. (20838300)
2010
29
Langerhans cell histiocytosis: fascinating dynamics of the dendritic cell-macrophage lineage. (20193021)
2010
30
Cranial unifocal Langerhans cell histiocytosis in children. (19302861)
2009
31
Cerebral non-Langerhans cell histiocytosis in a 17-year-old patient with a headache: case report. (19363626)
2009
32
A case of langerhans cell histiocytosis with anal fistula. (20505285)
2009
33
Immunocytochemical investigation of Langerin (CD207) is a valuable adjunct in the cytological diagnosis of Langerhans cell histiocytosis of the thyroid. (19195798)
2009
34
Langerhans cell histiocytosis: treatment failure with imatinib. (19687437)
2009
35
Intradural dirofilariasis mimicking a Langerhans cell histiocytosis tumor. (19489058)
2009
36
Langerhans cell histiocytosis with multifocal bone lesions: comparative clinical features between single and multi-systems. (19779766)
2009
37
Langerhans cell histiocytosis presenting as brown lichenoid patches. (20523803)
2009
38
Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations. (19218906)
2009
39
Aggressive Langerhans cell histiocytosis following T-ALL: clonally related neoplasms with persistent expression of constitutively active NOTCH1. (17874453)
2008
40
Concurrence of marginal zone B-cell lymphoma MALT-type and Langerhans cell histiocytosis in a thyroid gland with Hashimoto disease. (18401582)
2008
41
Anterior uveitis and iris nodules that are associated with Langerhans cell histiocytosis. (16376670)
2005
42
Serum levels of interleukin-1 receptor antagonist and tumor necrosis factor-alpha are elevated in children with Langerhans cell histiocytosis. (12794527)
2003
43
Differentiation of Langerhans cells in Langerhans cell histiocytosis. (11222366)
2001
44
Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus? (10698221)
2000
45
Detection of Langerhans cell histiocytosis lesions with somatostatin analogue scintigraphy--a preliminary report. (11070478)
2000
46
DNA polymorphisms and mutations of the tumor necrosis factor-alpha (TNF-alpha) promoter in Langerhans cell histiocytosis (LCH). (9355965)
1997
47
CNS sequelae in Langerhans cell histiocytosis: progressive spinocerebellar degeneration as a late manifestation of the disease. (9383811)
1997
48
Monokine expression in Langerhans' cell histiocytosis and sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) (8691347)
1996
49
Langerhans' cell histiocytosis. Definitive diagnosis with the use of monoclonal antibody O10 on routinely paraffin-embedded samples. (7755150)
1995
50
The hepatopulmonary syndrome masquerading as pulmonary Langerhans-cell histiocytosis. Hepatopulmonary Syndrome Study Group. (8198345)
1994

Variations for Langerhans-Cell Histiocytosis

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Expression for genes affiliated with Langerhans-Cell Histiocytosis

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Langerhans-Cell Histiocytosis

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Pathways for genes affiliated with Langerhans-Cell Histiocytosis

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Compounds for genes affiliated with Langerhans-Cell Histiocytosis

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GO Terms for genes affiliated with Langerhans-Cell Histiocytosis

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Products for genes affiliated with Langerhans-Cell Histiocytosis

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Langerhans-Cell Histiocytosis

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet