MCID: LNG024
MIFTS: 51

Langerhans-Cell Histiocytosis malady

Rare diseases, Respiratory diseases, Bone diseases, Blood diseases, Cancer diseases, Immune diseases categories

Aliases & Classifications for Langerhans-Cell Histiocytosis

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Sources:
9Disease Ontology, 41NIH Rare Diseases, 21Genetics Home Reference, 11DISEASES, 43Novoseek, 47Orphanet, 60UMLS, 55SNOMED-CT, 38NCIt, 33MeSH, 27ICD9CM, 34MESH via Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet
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Langerhans-Cell Histiocytosis, Aliases & Descriptions:

Name: Langerhans-Cell Histiocytosis 9 11
Letterer-Siwe Disease 9 41 11 47 60
Eosinophilic Granuloma 41 43 47 60
Histiocytosis X 9 41 21 43
Langerhans Cell Histiocytosis 41 21 43
Letterer-Siwe Disease of Lymph Nodes of Multiple Sites 9 60
Letterer-Siwe Disease of Intra-Abdominal Lymph Nodes 9 60
Letterer-Siwe Disease of Intrathoracic Lymph Nodes 9 60
Letterer-Siwe Disease of Intrapelvic Lymph Nodes 9 60
Lissencephaly Syndrome, Norman-Roberts Type 21 60
Lissencephaly with Cerebellar Hypoplasia 41 21
Letterer-Siwe Disease of Spleen 9 60
Langerhans Cell Granulomatosis 9 21
Hashimoto-Pritzger Disease 21 60
Lissencephaly 3 21 60
Acromelanosis 41 60
Lch 41 21
Letterer-Siwe Disease Involving Lymph Nodes of Inguinal Region and Lower Limb 9
Letterer-Siwe Disease of Lymph Nodes of Inguinal Region and/or Lower Limb 9
Letterer-Siwe Disease of Lymph Nodes of Inguinal Region Amd/or Lower Limb 9
Letterer-Siwe Disease of Lymph Nodes of Inguinal Region and Lower Limb 9
Letterer-Siwe Disease Involving Lymph Nodes of Axilla and Upper Limb 9
Letterer-Siwe Disease Involving Lymph Nodes of Head, Face, and Neck 9
 
Letterer-Siwe Disease Involving Lymph Nodes of Head, Face and Neck 9
Letterer-Siwe Disease of Lymph Nodes of Axilla and/or Upper Limb 9
Letterer-Siwe Disease of Lymph Nodes of Head, Face and/or Neck 9
Letterer-Siwe Disease Involving Lymph Nodes of Multiple Sites 9
Letterer-Siwe Disease of Lymph Nodes of Axilla and Upper Limb 9
Letterer-Siwe Disease Involving Intra-Abdominal Lymph Nodes 9
Letterer-Siwe Disease of Lymph Nodes of Head, Face and Neck 9
Letterer-Siwe Disease Involving Intrathoracic Lymph Nodes 9
Letterer-Siwe Disease Involving Intrapelvic Lymph Nodes 9
Acute and Disseminated Langerhans Cell Histiocytosis 47
Chronic and Multifocal Langerhans Cell Histiocytosis 47
Chronic and Localized Langerhans Cell Histiocytosis 47
Letterer-Siwe Disease Involving Spleen 9
Hand-Schüller-Christian Syndrome 41
Multifocal Eosinophilic Granuloma 47
Hand-Schüller-Christian Disease 47
Histiocytosis, Langerhans-Cell 60
Langerhan's Cell Histiocytosis 9
Norman-Roberts Syndrome 21
Lissencephaly 2 21
Lis2 21
Lis3 21


Classifications:



Characteristics (Orphanet epidemiological data):

47
letterer-siwe disease:
Prevalence: 1-9/1000000 (Europe); Age of onset: Infancy,Neonatal


External Ids:

Disease Ontology9 DOID:2571
ICD9CM27 202.5
Orphanet47 99871, 99873, 99870
MESH via Orphanet34 D004803, C538636
ICD10 via Orphanet26 C96.6, C96.5, C96.0
UMLS via Orphanet61 C0014461, C0023381

Summaries for Langerhans-Cell Histiocytosis

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NIH Rare Diseases:41 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 based summary: Langerhans-Cell Histiocytosis, also known as letterer-siwe disease, is related to histiocytosis and sarcoma, and has symptoms including bone pain, recurrent fractures and osteolysis. 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:9 A histiocytosis that is characterized by clonal 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.

Wikipedia:63 Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of Langerhans... more...

Related Diseases for Langerhans-Cell Histiocytosis

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

(show top 50)    (show all 206)
idRelated DiseaseScoreTop Affiliating Genes
1histiocytosis32.1CD1A, CD207
2sarcoma30.6CD207, CD1A
3langerhans cell sarcoma30.6CD207
4non-langerhans-cell histiocytosis10.9
5diabetes insipidus10.9
6erdheim-chester disease10.8
7juvenile xanthogranuloma10.8
8thyroiditis10.8
9leukemia10.7
10adult pulmonary langerhans cell histiocytosis10.7
11roberts syndrome10.7
12cervicitis10.6
13pneumothorax10.6
14rosai-dorfman disease10.6
15acromelanosis10.6
16central nervous system disease10.6
17nervous system disease10.6
18scabies10.6
19spontaneous pneumothorax10.6
20hodgkin lymphoma10.5
21dermatitis10.5
22mediastinitis10.5
23lissencephaly with cerebellar hypoplasia type f10.5
24lissencephaly with cerebellar hypoplasia type e10.5
25lissencephaly with cerebellar hypoplasia type b10.5
26lissencephaly with cerebellar hypoplasia type a10.5
27lissencephaly with cerebellar hypoplasia type d10.5
28lissencephaly with cerebellar hypoplasia type c10.5
29lissencephaly 310.5
30pulmonary hypertension10.4
31protein-losing enteropathy10.4
32cerebritis10.4
33sclerosing cholangitis10.4
34cholangitis10.4
35tuberculosis10.4
36pneumonia10.4
37lymphangioleiomyomatosis10.4
38acute leukemia10.4
39b-cell lymphomas10.4
40hemophagocytic lymphohistiocytosis10.4
41hydrocephalus10.4
42lymphoblastic lymphoma10.4
43marginal zone b-cell lymphoma10.4
44pulmonary fibrosis10.4
45dirofilariasis10.4
46tonsillitis10.4
47sensorineural hearing loss10.4
48folliculitis10.4
49adenocarcinoma10.4
50amyloidosis10.4

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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Symptoms:

 47 (show all 31)
  • 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
  • diabetes insipidus
  • follicular/erythematous/edematous papules/milium
  • hepatomegaly/liver enlargement (excluding storage disease)

HPO human phenotypes related to Langerhans-Cell Histiocytosis:

(show all 44)
id Description Frequency HPO Source Accession
1 bone pain hallmark (90%) HP:0002653
2 recurrent fractures hallmark (90%) HP:0002757
3 osteolysis hallmark (90%) HP:0002797
4 advanced eruption of teeth hallmark (90%) HP:0006288
5 chest pain hallmark (90%) HP:0100749
6 proptosis hallmark (90%) HP:0000520
7 diabetes insipidus hallmark (90%) HP:0000873
8 pulmonary infiltrates typical (50%) HP:0002113
9 abnormality of temperature regulation typical (50%) HP:0004370
10 otitis media occasional (7.5%) HP:0000388
11 proptosis occasional (7.5%) HP:0000520
12 hyperhidrosis occasional (7.5%) HP:0000975
13 tapered finger occasional (7.5%) HP:0001182
14 joint swelling occasional (7.5%) HP:0001386
15 migraine occasional (7.5%) HP:0002076
16 restrictive lung disease occasional (7.5%) HP:0002091
17 pulmonary hypertension occasional (7.5%) HP:0002092
18 respiratory insufficiency occasional (7.5%) HP:0002093
19 emphysema occasional (7.5%) HP:0002097
20 lymphoma occasional (7.5%) HP:0002665
21 lymphadenopathy occasional (7.5%) HP:0002716
22 decreased body weight occasional (7.5%) HP:0004325
23 hemiplegia/hemiparesis occasional (7.5%) HP:0004374
24 chronic obstructive pulmonary disease occasional (7.5%) HP:0006510
25 neoplasm of the lung occasional (7.5%) HP:0100526
26 abnormality of the skin occasional (7.5%) HP:0000951
27 pulmonary infiltrates occasional (7.5%) HP:0002113
28 hepatomegaly occasional (7.5%) HP:0002240
29 autosomal recessive inheritance HP:0000007
30 irritability HP:0000737
31 abnormality of the skeletal system HP:0000924
32 jaundice HP:0000952
33 pallor HP:0000980
34 seborrheic dermatitis HP:0001051
35 encephalopathy HP:0001298
36 hepatosplenomegaly HP:0001433
37 thrombocytopenia HP:0001873
38 neutropenia HP:0001875
39 anemia HP:0001903
40 fever HP:0001945
41 dyspnea HP:0002094
42 pulmonary infiltrates HP:0002113
43 abdominal distention HP:0003270
44 stomatitis HP:0010280

Drugs & Therapeutics for Langerhans-Cell Histiocytosis

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Drug clinical trials:

Search ClinicalTrials for Langerhans-Cell Histiocytosis

Search NIH Clinical Center for Langerhans-Cell Histiocytosis

Inferred drug relations via UMLS60/NDF-RT39:

Genetic Tests for Langerhans-Cell Histiocytosis

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Anatomical Context for Langerhans-Cell Histiocytosis

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MalaCards organs/tissues related to Langerhans-Cell Histiocytosis:

31
Lymph node, Bone, Spleen, Lung, Skin, Bone marrow, Liver, Thyroid, Pituitary, Myeloid, T cells, Tongue, Thymus, Colon, Breast, Testes, Spinal cord, B cells, Cortex, Heart, Tonsil, Monocytes

Animal Models for Langerhans-Cell Histiocytosis or affiliated genes

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Publications for Langerhans-Cell Histiocytosis

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Articles related to Langerhans-Cell Histiocytosis:

(show top 50)    (show all 681)
idTitleAuthorsYear
1
Langerhans cell histiocytosis in an adult with involvement of the calvarium, cerebral cortex and brainstem: discussion of pathophysiology and rationale for the use of intravenous immune globulin. (25873887)
2015
2
Severe pulmonary hypertension in adult pulmonary Langerhans cell histiocytosis: the effect of sildenafil as a bridge to lung transplantation. (25175135)
2014
3
Antiphospholipid syndrome and Langerhans cell histiocytosis: An exceptional association. (25867157)
2014
4
Chromium-Induced Diffuse Dermatitis and Lymph Node Involvement by Langerhans Cell Histiocytosis After Metal-on-Metal Hip Resurfacing. (25385406)
2014
5
Pulmonary Langerhans cell histiocytosis in children: A spectrum of radiologic findings. (23769766)
2014
6
Skeletal involvement in Langerhans cell histiocytosis. (23907805)
2013
7
Rapid resolution of pulmonary Langerhans cell histiocytosis. (23749831)
2013
8
Differentiating pulmonary lymphangioleiomyomatosis from pulmonary langerhans cell histiocytosis and Birt-Hogg-Dube syndrome. (24339506)
2013
9
Pulmonary adenocarcinoma in a young patient of pulmonary langerhans cell histiocytosis (PLCH). (23857403)
2013
10
Multisystem Langerhans cell histiocytosis in adult. (22470214)
2012
11
Juvenile xanthogranuloma developing after treatment of Langerhans cell histiocytosis: case report and literature review. (22977671)
2012
12
Langerhans' cell histiocytosis of the temporal bone. (21725272)
2012
13
Langerhans cell histiocytosis of the temporal bone. (21970849)
2012
14
Adult Langerhans cell histiocytosis in a middle aged Ethiopian woman. (22924288)
2012
15
Pulmonary langerhans cell histiocytosis. (22429393)
2012
16
Langerhans cell histiocytosis: two clinical presentations in the same patient. (22301045)
2012
17
Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy. (22835048)
2012
18
Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: a case of extramedullary hematopoiesis. (21922646)
2012
19
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
20
Treatment of patients with hypothalamic-pituitary lesions as adult-onset Langerhans cell histiocytosis. (22015494)
2011
21
Langerhans cell histiocytosis preceding the development of juvenile xanthogranuloma: a case and review of recent developments. (21793710)
2011
22
Localized Langerhans cell histiocytosis of the stomach treated by endoscopic submucosal dissection. (21837608)
2011
23
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
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
Langerhans cell histiocytosis with thyroid and lung involvement in a child: a case report. (20224443)
2010
27
Langerhans cell histiocytosis in an adult: good response of cutaneous lesions to acitretin. (20184614)
2010
28
MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis: experience on seven patients. (19861578)
2010
29
Multifocal Langerhans cell histiocytosis with infiltrative pelvic lesions: PET/CT imaging. (20838300)
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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Search GEO for disease gene expression data for Langerhans-Cell Histiocytosis.

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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Sources for Langerhans-Cell Histiocytosis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet