LCH
MCID: LNG024
MIFTS: 63

Langerhans-Cell Histiocytosis (LCH) malady

Respiratory, Endocrine, Blood, Cancer categories

Summaries for Langerhans-Cell Histiocytosis

Sources:
8Disease Ontology, 21Genetics Home Reference, 43NIH Rare Diseases, 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 juvenile xanthogranuloma and acute leukemia, and has symptoms including anomalies of tongue, gingiva and oral mucosa, anomalies of teeth and dentition and premature eruption of teeth/natal teeth. An important gene associated with Langerhans-Cell Histiocytosis is F13A1 (coagulation factor XIII, A1 polypeptide), and among its related pathways are Malaria and LDL Oxidation in Atherogenesis. The drug thalidomide and the compounds butyrate and estrogen have been mentioned in the context of this disorder. Related mouse phenotypes are muscle and skeleton.

Disease Ontology:8 A disease of metabolism and histiocytic and dendritic cell cancer and histiocytosis and interstitial lung disease that results in the proliferation of langerhans cells.

Genetics Home Reference:21 Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin, lymph nodes, spleen, lungs, liver, and bone marrow. In Langerhans cell histiocytosis, excess immature Langerhans cells usually form tumors called granulomas. However, Langerhans cell histiocytosis is not generally considered to be a form of cancer.

Description from OMIM:47 604856

Aliases & Classifications for Langerhans-Cell Histiocytosis

Sources:
43NIH Rare Diseases, 8Disease Ontology, 10DISEASES, 21Genetics Home Reference, 45Novoseek, 49Orphanet, 47OMIM, 61UMLS, 40NCIt, 57SNOMED-CT, 35MeSH, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer
Anatomical: Respiratory, Endocrine, Blood


Characteristics (Orphanet epidemiological data):

49
langerhans cell histiocytosis:
Prevalence: 1-9/100000; Age of onset: Variable; Age of death: Normal


Aliases & Descriptions:

langerhans-cell histiocytosis 8 10
langerhans cell histiocytosis 43 21 45 49 47
histiocytosis x 8 43 21 45 49
eosinophilic granuloma 43 45 49 61
langerhans cell granulomatosis 8 21 49
lch 43 21
chronic and localized langerhans cell histiocytosis 49
hand-schüller-christian syndrome 43
histiocytosis, langerhans-cell 61
langerhan's cell histiocytosis 8
hashimoto-pritzger disease 21
letterer-siwe disease 43


External Ids:

Disease Ontology8 DOID:2571
NCIt40 C3107, C6920
MeSH35 D006646
OMIM47 604856
MESH via Orphanet36 D006646, D004803
ICD10 via Orphanet26 C96.0, C96.5, C96.6
SNOMED-CT via Orphanet58 128809007, 190955000, 65399007 129000002, more
UMLS via Orphanet62 C0019621, C0014461

Related Diseases for Langerhans-Cell Histiocytosis

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Langerhans-Cell Histiocytosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 290)
idRelated DiseaseScoreTop Affiliating Genes
1juvenile xanthogranuloma31.2FSCN1, F13A1, S100B
2acute leukemia30.9PML, CSF1, FLT3LG, RARA
3rosai-dorfman disease30.9FSCN1, CD163, CTSD, S100B
4langerhans cell sarcoma30.8CD207
5letterer-siwe disease30.7CD1A, CD207
6amyloidosis30.5CTSD
7adenocarcinoma30.5CTSD, F13A1, LIF, FSCN1, RARA, ACTC1
8idiopathic pulmonary fibrosis30.5CCL22, S100A1, ACTC1
9aspergillosis30.3CSF1
10fibromatosis30.3S100A1, ACTC1
11acute monocytic leukemia30.3RARA, CD40, CSF1
12anaplastic large cell lymphoma30.3FSCN1
13stomach cancer30.2CTSD, LIF, FSCN1, RARA
14fibrous histiocytoma30.2S100B, F13A1, CD163, FSCN1, ACTC1
15asthma30.2CCL22, CSF1, TNF, LIF, CD1A, IL10
16thrombocytopenia30.2FLT3LG, CSF1
17squamous cell carcinoma30.2S100A1, CTSD, LIF, FSCN1, RARA, ACTC1
18thyroid cancer30.2S100A1, CTSD, RARA
19synovitis30.2TNF, CD163, CD68
20reticulosarcoma30.0FSCN1, CD68, CTSD
21graves' disease30.0CD40
22diabetes insipidus10.8
23erdheim-chester disease10.8
24adult pulmonary langerhans cell histiocytosis10.7
25lip disease10.6
26spontaneous pneumothorax10.5
27pulmonary function10.5
28papillary carcinoma10.5
29acute myeloid leukemia10.4
30protein-losing enteropathy10.4
31sclerosing cholangitis10.4
32t-cell leukemia10.4
33tuberculosis10.4
34sensorineural hearing loss10.3
35lymphangioleiomyomatosis10.3
36pulmonary sarcoidosis10.3
37cellulitis10.3
38acute t cell leukemia10.3
39dirofilariasis10.3
40malignant histiocytosis10.3
41n syndrome10.3
42spinocerebellar degeneration10.3
43hydrops fetalis10.3
44precocious puberty10.3
45lichen sclerosus10.3
46lymphoblastic lymphoma10.3
47ataxia10.3
48langerhans cell histiocytosis in childhood and adulthood10.3
49langerhans cell histiocytosis specific to childhood10.3
50langerhans cell histiocytosis specific to adulthood10.3

Graphical network of the top 20 diseases related to Langerhans-Cell Histiocytosis:



Diseases related to langerhans-cell histiocytosis

Clinical Features for Langerhans-Cell Histiocytosis

Sources:
47OMIM, 49Orphanet
See all sources

Clinical features from OMIM:

604856

Symptoms:

49 (show all 28)
  • anomalies of tongue, gingiva and oral mucosa
  • anomalies of teeth and dentition
  • premature eruption of teeth/natal teeth
  • thoracic/chest pain
  • mutiple fractures/bone fragility
  • osteolysis/osteoclasia/bone destruction/erosions
  • bone pain
  • lung/pulmonary infiltrates
  • bone tumefaction/swelling
  • fever/chilling
  • asthenia/fatigue/weakness
  • proptosis/exophthalmos
  • chronic/relapsing otitis
  • tapered fingers
  • hyperhidrosis/increased sweating
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • emphysema
  • cough
  • pulmonary hypertension
  • interstitial/restrictive pneumopathy/restrictive respiratory syndrome
  • chronic obstructive pulmonary disease/copd/obstructive respiratory syndrome
  • lymphadenopathy/polyadenopathies
  • facial pain/cephalalgia/migraine
  • hemiplegia/diplegia/hemiparesia/limb palsy
  • hydrarthrosis/articular/joint effusion
  • lung/bronchopulmonary neoplasm/tumor/carcinoma/cancer
  • lymphoma
  • wasted (excluding lipodystrophy)/poorly muscled build/cachexy

Drugs & Therapeutics for Langerhans-Cell Histiocytosis

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Langerhans-Cell Histiocytosis

Drug clinical trials:

Search ClinicalTrials for Langerhans-Cell Histiocytosis

Search NIH Clinical Center for Langerhans-Cell Histiocytosis

Search CenterWatch for Langerhans-Cell Histiocytosis

Inferred drug relations via UMLS61/NDF-RT41:

Genetic Tests for Langerhans-Cell Histiocytosis

Anatomical Context for Langerhans-Cell Histiocytosis

Animal Models for Langerhans-Cell Histiocytosis or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Langerhans-Cell Histiocytosis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000536910.2CSF1, RARA, ACTC1
2MP:000539010.2RARA, CD40, CD68, LIF, CSF1
3MP:00053979.9RARA, PML, CSF1, TNF, CTSD, FLT3LG
4MP:00053849.9PML, CSF1, TNF, CTSD, FLT3LG, LIF
5MP:00053879.8FLT3LG, CTSD, TNF, CSF1, PML, LIF
6MP:00053859.8S100A1, CSF1, TNF, F13A1, LIF, IL10

Publications for Langerhans-Cell Histiocytosis

Sources:
51PubMed
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Articles related to Langerhans-Cell Histiocytosis:

(show top 50)    (show all 646)
idTitleAuthorsYear
1
Langerhans cell histiocytosis associated with lichen sclerosus of the vulva: case report and review of the literature. (23167438)
2013
2
Spontaneous remission of 2-chlorodeoxyadenosine (2-CdA)-related secondary myelodysplastic syndrome in a patient with refractory Langerhans cell histiocytosis. (23616222)
2013
3
Intriguing bronchoalveolar lavage proteome in a case of pulmonary langerhans cell histiocytosis. (23875058)
2013
4
Utility of bronchoscopy in pulmonary Langerhans cell histiocytosis. (24162113)
2013
5
Unilesional self-limited Langerhans cell histiocytosis: a case report and review of the literature. (23550725)
2013
6
Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. (23589673)
2013
7
Radiological features of thymic langerhans cell histiocytosis. (23813898)
2013
8
Langerhans cell histiocytosis case with dense metaphyseal band sign. (23409985)
2013
9
Clinical Features and Treatment Outcomes of Langerhans Cell Histiocytosis: A Nationwide Survey From Korea Histiocytosis Working Party. (24276037)
2013
10
Juvenile xanthogranuloma developing after treatment of Langerhans cell histiocytosis: case report and literature review. (22977671)
2012
11
Langerhans' cell histiocytosis of the temporal bone. (21725272)
2012
12
Coexistence of Langerhans cell histiocytosis, Rosai-Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver. (23043641)
2012
13
Multisystem Langerhans cell histiocytosis with liver dysfunction as the first presentation: A case report. (22740918)
2012
14
An exploratory epidemiological study of Langerhans cell histiocytosis. (22434707)
2012
15
Management of adult primary vulvar Langerhans cell histiocytosis: review of the literature and a case history. (22464205)
2012
16
B-RAF mutant alleles associated with Langerhans cell histiocytosis, a granulomatous pediatric disease. (22506009)
2012
17
Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension: clinical characteristics and impact of pulmonary arterial hypertension therapies. (22459770)
2012
18
Rapidly progressing fatal adult multi-organ Langerhans cell histiocytosis complicated with fatty liver disease. (23037628)
2012
19
Congenital Langerhans cell histiocytosis involving only one of non-identical twins. (21700531)
2011
20
The Langerhans cell histiocytosis: a disease in search of an identity. (23049335)
2011
21
DTI findings of brainstem involvement in Langerhans' cell histiocytosis. (20844850)
2011
22
Langerhans cell histiocytosis treated with narrow-band ultraviolet B. (21269310)
2011
23
Spontaneous pneumothorax in a case of pulmonary langerhans cell histiocytosis. (22368698)
2011
24
Uncommon features of pulmonary Langerhans' cell histiocytosis: analysis of 11 cases and a review of the literature. (20193493)
2010
25
Cutaneous Langerhans cell histiocytosis in an elderly woman. (21062600)
2010
26
A case of Langerhans cell histiocytosis in an adult with initial symptoms confined to the skin. (20465678)
2010
27
Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus treated with vincristine/cytosine arabinoside. (19908293)
2010
28
Multisystem langerhans cell histiocytosis with advanced orbital involvement: case report. (23057277)
2010
29
Simultaneous Occurrence of Hodgkin's Lymphoma and Langerhans Cell Histiocytosis of the Spine : A Rare Combination. (20461174)
2010
30
Langerhans cell histiocytosis of the uvea with neovascular glaucoma: diagnosis by fine-needle aspiration biopsy and management with intraocular bevacizumab and brachytherapy. (21168078)
2010
31
Chromosomal integration of the HHV-6 genome as a possible cause of persistent HHV-6 detection in a patient with langerhans cell histiocytosis. (19731091)
2010
32
Detection of clonal lymphoid receptor gene rearrangements in langerhans cell histiocytosis. (20551822)
2010
33
Treatment of adult-onset Langerhans cell histiocytosis--is it different from the pediatric approach? (19940006)
2010
34
Hypopigmented scaly, scalp and facial lesions and disfiguring exopthalmus. Langerhans cell histiocytosis (LCH). (20500179)
2010
35
Unifocal Granuloma of Femur due to Langerhans' Cell Histiocytosis: A Case Report and Review of the Literature. (20811571)
2010
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
Interleukin-17A is not expressed by CD207(+) cells in Langerhans cell histiocytosis lesions. (19424201)
2009
39
Langerhans cell histiocytosis: malignancy or inflammatory disorder doing a great job of imitating one? (19726802)
2009
40
Langerhans cell histiocytosis associated with breast carcinoma successfully treated with topical imiquimod. (19843082)
2009
41
Development of Langerhans cell histiocytosis associated with chronic active Epstein-Barr virus infection. (17474115)
2008
42
Cerebellar ataxia in pediatric patients with Langerhans cell histiocytosis. (15543008)
2004
43
Disseminated BCG infection resembling langerhans cell histiocytosis in an infant with severe combined immunodeficiency: a case report. (15552821)
2004
44
Tumor necrosis factor, interleukin 11, and leukemia inhibitory factor produced by Langerhans cells in Langerhans cell histiocytosis. (15543003)
2004
45
Sinus histiocytosis with massive lymphadenopathy and Langerhans cell histiocytosis express the cellular adhesion molecule CD31. (12653580)
2003
46
Langerhans cell histiocytosis immunohistochemical expression of fascin, a dendritic cell marker. (12219775)
2002
47
Problems in differential diagnosis of non Langerhans cell histiocytosis with pituitary involvement: case report and review of literature. (12665654)
2002
48
Hypopituitarism in langerhans cell histiocytosis: seven cases and literature review. (11686544)
2001
49
Juvenile xanthogranuloma versus Langerhans cell histiocytosis (histiocytosis X). (10188845)
1999
50
Elevated erythrocyte sedimentation rate and thrombocytosis as possible indicators of active disease in Langerhans' cell histiocytosis. (9825978)
1998

Genetic Variations for Langerhans-Cell Histiocytosis

Expression for genes affiliated with Langerhans-Cell Histiocytosis

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Langerhans-Cell Histiocytosis

Search GEO for disease gene expression data for Langerhans-Cell Histiocytosis.

Pathways for genes affiliated with Langerhans-Cell Histiocytosis

Sources:
30KEGG, 52QIAGEN
See all sources

Compounds for genes affiliated with Langerhans-Cell Histiocytosis

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 29IUPHAR, 24HMDB, 60Tocris Bioscience, 2BitterDB
See all sources

Compounds related to Langerhans-Cell Histiocytosis according to GeneCards/GeneDecks:

(show top 50)    (show all 93)
idCompoundScoreTop Affiliating Genes
1butyrate4510.8CD40, CSF1
2estrogen4510.8F13A1
3cyclophosphamide45 50 1112.7FLT3LG, CD40
4gm-csf45 2911.7LIF, IL10, TNF
5s 1004510.7S100B, F13A1, ACTC1
6ivig4510.7CD40, CSF1
7reticulin4510.7ACTC1
8glycogen45 2411.7LIF, CSF1, ACTC1
9rantes4510.7CD40, LIF, CSF1, TNF
10dexamethasone45 50 29 1113.7S100B, LIF, CD163, RARA
11b 7234510.7ACTC1, S100A1, S100B, F13A1
12polyinosinic-polycytidylic acid4510.7CSF1, TNF, CD40, IL10
13il 104510.7CSF1, TNF, LIF, CD40, IL10
14blys4510.7TNF, IL10, CD40
15biotin45 11 2412.6S100A1, CSF1, CD40, IL10, ACTC1
16paclitaxel45 50 1112.6CSF1, RARA, LIF, CTSD, S100B
17prostaglandin4510.6CSF1, CD40, CCL22, TNF
18mannose4510.6CD207, CD40, CTSD, CSF1, CD163
19tgf beta14510.6TNF, LIF, RARA, IL10, CSF1
20hematoxylin4510.6S100B, ACTC1, F13A1, S100A1
21mucicarmine4510.6ACTC1, S100A1, S100B
22imatinib45 50 1112.6S100B, CSF1, ACTC1, RARA, IL10
23mdms4510.6S100B, CSF1, IL10
24ionomycin4510.6S100B, CSF1, TNF, IL10, CD40
25calcitriol45 60 11 2413.6IL10, LIF, CTSD, TNF, CSF1, RARA
26creatinine4510.6ACTC1, CTSD, CSF1
27neopterin4510.6CD40, TNF, IL10, CSF1
28rituximab45 50 1112.6CD40, TNF, CSF1, IL10
2912-o-tetradecanoylphorbol 13-acetate4510.6CSF1, CTSD, LIF, CD40, FSCN1, RARA
30actinomycin d4510.5LIF, PML, CSF1, TNF, CD40, RARA
31cyclosporin a45 29 6012.5CD40, ACTC1, CCL22, S100B, TNF, CTSD
32p0034510.5S100B, CSF1, CTSD
33alpha-retinoic acid4510.5PML, RARA
34progesterone45 60 29 11 2414.5S100B, S100A1, CSF1, CTSD, F13A1, LIF
35etoposide45 50 60 1113.5PML, CTSD, S100B, RARA, CSF1
36nitric oxide45 11 2412.5IL10, CD40, RARA, LIF, CTSD, TNF
37thymidine45 2411.4CSF1, IL10, RARA, LIF, FLT3LG, S100B
38sb 20358045 6011.4CCL22, TNF, IL10, LIF, CD40
39cysteine4510.4CD40, RARA, S100A1, CSF1, PML, S100B
40vegf4510.4LIF, F13A1, FLT3LG, CTSD, CD40, IL10
41hydrocortisone45 2 60 1113.3LIF
42retinoic acid45 2411.3ACTC1, FSCN1, CSF1, TNF, CTSD, LIF
43fibrinogen4510.3CD40, F13A1, CCL22, LIF, TNF
44oxygen45 2411.3TNF, CD40, S100B, CTSD
45lipid4510.3CD163, S100B, RARA, CD40, CD1A, CD68
46prednisolone45 29 1112.3IL10, CD40, TNF, CSF1
47paraffin4510.2S100B, ACTC1, S100A1, CSF1, CTSD, F13A1
48calcium45 50 11 2413.2F13A1, S100A1, ACTC1, RARA, FSCN1, CD101
49tyrosine4510.2S100A1, CSF1, ACTC1, LIF, FSCN1, S100B
50histamine45 29 2411.9IL10, CSF1, CCL22, TNF, CD40

GO Terms for genes affiliated with Langerhans-Cell Histiocytosis

Sources:
16Gene Ontology
See all sources

Cellular components related to Langerhans-Cell Histiocytosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:00557610.1S100B, TNF, CTSD, F13A1, CD163, CD40
2extracellular spaceGO:0056159.7IL10, CCL22, S100B, CSF1, TNF, CTSD

Biological processes related to Langerhans-Cell Histiocytosis according to GeneCards/GeneDecks:

(show all 11)
idNameGO IDScoreTop Affiliating Genes
1receptor biosynthetic processGO:03280010.6TNF, IL10
2negative regulation of cytokine secretion involved in immune responseGO:00274010.6IL10, TNF
3regulation of immunoglobulin secretionGO:05102310.6CD40, TNF
4positive regulation of tyrosine phosphorylation of Stat1 proteinGO:04251110.5CD40, LIF
5inflammatory responseGO:00695410.5IL10, CD40, TNF, CSF1, CCL22
6response to glucocorticoid stimulusGO:05138410.4IL10, TNF, S100B
7positive regulation of macrophage differentiationGO:04565110.4CSF1, LIF
8branching involved in mammary gland duct morphogenesisGO:06044410.4CSF1, PML
9positive regulation of cell proliferationGO:00828410.4S100B, CSF1, FLT3LG, LIF, RARA
10B cell proliferationGO:04210010.3IL10, CD40
11retinoic acid receptor signaling pathwayGO:04838410.1RARA, PML

Molecular functions related to Langerhans-Cell Histiocytosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein homodimerization activityGO:04280310.2FLT3LG, CSF1, S100A1, S100B, PML
2cytokine activityGO:00512510.1IL10, LIF, FLT3LG, TNF, CSF1
3S100 protein bindingGO:04454810.1S100B, S100A1

Products for genes affiliated with Langerhans-Cell Histiocytosis

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Sources for Langerhans-Cell Histiocytosis

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet