MCID: HST010
MIFTS: 63

Histiocytosis malady

Rare diseases, Immune diseases categories

Summaries for Histiocytosis

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Wikipedia:64 In medicine, histiocytosis refers to an excessive number of histiocytes, (tissue macrophages), and is... more...

MalaCards based summary: Histiocytosis, also known as hand-schueller-christian syndrome, is related to langerhans-cell histiocytosis and rosai-dorfman disease. An important gene associated with Histiocytosis is F13A1 (coagulation factor XIII, A1 polypeptide), and among its related pathways are Signaling events mediated by PTP1B and Integrins in angiogenesis. The drugs etoposide and vinblastine and the compounds mdms and aclarubicin have been mentioned in the context of this disorder. Affiliated tissues include bone, t cells and bone marrow, and related mouse phenotypes are integument and cardiovascular system.

Disease Ontology:9 A lymphatic system disease that is characterized by an excessive number of histiocytes.

Aliases & Classifications for Histiocytosis

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Histiocytosis, Aliases & Descriptions:

Name: Histiocytosis 9 11 44 61
Hand-Schueller-Christian Syndrome 61
Hand-Schuller-Christian Syndrome 42
Hand-Schueller-Christian Disease 61
 
Hand Schuller Christian Disease 9
Hand-Schuller-Christian Disease 42
Histiocytosis, Langerhans-Cell 61
Chronic Histiocytosis X 9


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Immune diseases


External Ids:

Disease Ontology9 DOID:3405
MeSH34 D015614
NCIt39 C3106

Related Diseases for Histiocytosis

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Diseases in the Histiocytosis family:

Malignant Histiocytosis

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

(show top 50)    (show all 330)
idRelated DiseaseScoreTop Affiliating Genes
1langerhans-cell histiocytosis32.4F13A1, S100B
2rosai-dorfman disease31.8S100B
3malignant histiocytosis31.6MYB, CSF1R, CSF1
4juvenile xanthogranuloma31.3F13A1, S100B
5langerhans cell sarcoma30.9CD207
6monocytic leukemia30.3LIF, CSF1
7leukemia30.3MYB, LIF, CSF1R, CSF1, CD1A
8arthritis30.1CSF1, CSF1R, LIF
9fibrous histiocytoma30.1F13A1, ACTC1, S100B
10acute myelomonocytic leukemia30.1CSF1, CSF1R
11acute leukemia30.0CSF1R, MYB, CSF1
12rheumatoid arthritis30.0CSF1, LIF, MYB, F13A1
13teratoma30.0ACTC1, LIF
14papilloma29.7ACTC1
15myeloid leukemia29.7MYB, CSF1R, CSF1, LIF
16osteoarthritis29.5F13A1, LIF, CSF1R, CSF1
17sarcoma29.5CD207, ACTC1, CSF1R, CSF1, CD1A, S100B
18glioblastoma29.3MYB, S100B, CSF1R
19ipex syndrome29.2LIF, MYB, CD207, S100B, CSF1
20adenocarcinoma29.1F13A1, MYB, ACTC1, CSF1R, LIF
21sea-blue histiocytosis10.6
22benign cephalic histiocytosis10.6
23non-langerhans-cell histiocytosis10.6
24indeterminate cell histiocytosis10.6
25erdheim-chester disease10.5
26diabetes insipidus10.5
27perineurioma10.5S100B
28thyroiditis10.5
29ganglioglioma10.4S100B
30generalized eruptive histiocytosis10.4
31progressive nodular histiocytosis10.4
32histiocytosis-lymphadenopathy plus syndrome10.4
33b-cell lymphomas10.4
34hereditary progressive mucinous histiocytosis10.4
35adult pulmonary langerhans cell histiocytosis10.3
36niemann-pick disease10.3
37mycosis fungoides10.3CD1A, F13A1
38mediastinitis10.3
39virus associated hemophagocytic syndrome10.3
40paraganglioma10.3ACTC1
41gaucher's disease10.3
42cervicitis10.3
43histiocytoma10.3
44pneumothorax10.3
45histiocytic sarcoma10.2
46pigmented villonodular synovitis10.2CSF1, CSF1R
47tuberous sclerosis10.2F13A1, S100B
48hodgkin's lymphoma10.2
49marginal zone b-cell lymphoma10.2
50alk+ histiocytosis10.2

Graphical network of the top 20 diseases related to Histiocytosis:



Diseases related to histiocytosis

Symptoms for Histiocytosis

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Drugs & Therapeutics for Histiocytosis

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Drug clinical trials:

Search ClinicalTrials for Histiocytosis

Search NIH Clinical Center for Histiocytosis

Inferred drug relations via UMLS61/NDF-RT40:

Genetic Tests for Histiocytosis

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Anatomical Context for Histiocytosis

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MalaCards organs/tissues related to Histiocytosis:

32
Bone, T cells, Bone marrow, Skin, Thyroid, Lung, B cells, Liver, Myeloid, Lymph node, Monocytes, Pituitary, Spleen, Brain, Colon, Pancreas, Spinal cord, Breast, Testes, Thymus, Small intestine, Tonsil, Tongue, Eye, Heart, Temporal lobe, Hypothalamus, Neutrophil

Animal Models for Histiocytosis or affiliated genes

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MGI Mouse Phenotypes related to Histiocytosis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107717.8S100B, MYB, LIF, CSF1R, CSF1
2MP:00053857.8CSF1, LIF, MYB, F13A1, ACTC1
3MP:00053877.7CSF1, CSF1R, LIF, SLC29A3, MYB, CD207
4MP:00053977.5F13A1, MYB, LIF, CSF1R, CSF1, CD207
5MP:00107686.9SLC29A3, F13A1, CSF1, CSF1R, ACTC1, LIF
6MP:00053846.9CSF1R, ACTC1, LIF, MYB, CSF1, SLC29A3

Publications for Histiocytosis

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Articles related to Histiocytosis:

(show top 50)    (show all 1354)
idTitleAuthorsYear
1
Severe pulmonary hypertension in adult pulmonary Langerhans cell histiocytosis: the effect of sildenafil as a bridge to lung transplantation. (25175135)
2014
2
Skeletal involvement in Langerhans cell histiocytosis. (23907805)
2013
3
Rapid resolution of pulmonary Langerhans cell histiocytosis. (23749831)
2013
4
Multisystem Langerhans cell histiocytosis in adult. (22470214)
2012
5
Rosai-dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: third reported occurrence. (22864941)
2012
6
Juvenile xanthogranuloma developing after treatment of Langerhans cell histiocytosis: case report and literature review. (22977671)
2012
7
Concomitant occurrence of sinus histiocytosis with massive lymphadenopathy and nodal marginal zone lymphoma. (21366466)
2011
8
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
9
Treatment of patients with hypothalamic-pituitary lesions as adult-onset Langerhans cell histiocytosis. (22015494)
2011
10
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
11
Uncommon features of pulmonary Langerhans' cell histiocytosis: analysis of 11 cases and a review of the literature. (20193493)
2010
12
Cutaneous Langerhans cell histiocytosis in an elderly woman. (21062600)
2010
13
A role for ITPA variants in the clinical course of pulmonary Langerhans' cell histiocytosis? (20930204)
2010
14
Langerhans cell histiocytosis with thyroid and lung involvement in a child: a case report. (20224443)
2010
15
A case of a Korean adult affected by type B Niemann-Pick disease: secondary sea-blue histiocytosis and molecular characterization]. (19411774)
2009
16
Japanese case of benign cephalic histiocytosis. (19207442)
2009
17
Cranial unifocal Langerhans cell histiocytosis in children. (19302861)
2009
18
Cerebral non-Langerhans cell histiocytosis in a 17-year-old patient with a headache: case report. (19363626)
2009
19
Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: second case report. (19020944)
2009
20
A case of langerhans cell histiocytosis with anal fistula. (20505285)
2009
21
Aggressive Langerhans cell histiocytosis following T-ALL: clonally related neoplasms with persistent expression of constitutively active NOTCH1. (17874453)
2008
22
Progressive nodular histiocytosis - A five-year follow up. (18424392)
2008
23
Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease): evidence for a dendritic cell derivation? (16761737)
2005
24
Indeterminate cell histiocytosis: a case report. (15481563)
2004
25
Primary histiocytic sarcoma of the spleen associated with erythrophagocytic histiocytosis. (12747473)
2003
26
Serum levels of interleukin-1 receptor antagonist and tumor necrosis factor-alpha are elevated in children with Langerhans cell histiocytosis. (12794527)
2003
27
Extranodal sinus histiocytosis (Rosai-Dorfman disease) of the brain parenchyma. (12601463)
2003
28
Sinus histiocytosis with massive lymphadenopathy: complicated by autoimmune hemolytic anemia. (14620195)
2003
29
Extranodal marginal zone B-cell lymphoma of the lacrimal gland associated with crystal-storing histiocytosis. (11772588)
2002
30
Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus? (10698221)
2000
31
Detection of Langerhans cell histiocytosis lesions with somatostatin analogue scintigraphy--a preliminary report. (11070478)
2000
32
Indeterminate-cell histiocytosis: immunophenotypic and cytogenetic findings in an infant. (10102017)
1999
33
DNA polymorphisms and mutations of the tumor necrosis factor-alpha (TNF-alpha) promoter in Langerhans cell histiocytosis (LCH). (9355965)
1997
34
Modulation of fibroblast activity in histiocytosis X by platelet-derived growth factor. (7874940)
1995
35
Malignant histiocytosis in childhood: a distinctive CD30-positive clinicopathological entity associated with a chromosomal translocation involving 5q35. (1313990)
1992
36
Benign cephalic histiocytosis: a case report. (2400727)
1990
37
Malignant histiocytosis presenting as erythroderma. (2139870)
1990
38
Distribution of three proteinases in tumor cells of malignant histiocytosis]. (2279308)
1990
39
An immunocytochemical study on the distribution of ferritin and other markers in 36 cases of malignant histiocytosis. (2670186)
1989
40
Chronic myelomonocytic leukemia terminating as malignant histiocytosis. (3334348)
1987
41
Malignant histiocytosis complicating rheumatoid arthritis: report of four cases. (3950028)
1986
42
Chromosomal and fluorometric DNA analyses of malignant histiocytosis. (3811786)
1986
43
Histiocytosis X and erythropoietic protoporphyria. (6607997)
1984
44
Erythema annulare centrifugum and malignant histiocytosis--report of a case. (6499278)
1984
45
Malignant histiocytosis. Clinical and cytogenetic studies in a newborn and a child. (6883287)
1983
46
Non-B, non-T acute lymphoblastic leukaemia terminating in malignant histiocytosis: a new case and a review of the literature. (6293942)
1982
47
Ichthyosis and marrow involvement in malignant histiocytosis of the intestine. (7236527)
1981
48
Malignant histiocytosis in childhood: clinical study and therapeutic results in 22 cases. (7379013)
1980
49
Malignant histiocytosis in children from the Eastern Mediterranean. (7453672)
1980
50
Malignant histiocytosis associated with malabsorption. (262066)
1979

Variations for Histiocytosis

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Expression for genes affiliated with Histiocytosis

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Expression patterns in normal tissues for genes affiliated with Histiocytosis

Search GEO for disease gene expression data for Histiocytosis.

Pathways for genes affiliated with Histiocytosis

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Compounds for genes affiliated with Histiocytosis

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Compounds related to Histiocytosis according to GeneCards/GeneDecks:

(show top 50)    (show all 68)
idCompoundScoreTop Affiliating Genes
1mdms449.8CSF1, S100B
2aclarubicin449.7MYB, CSF1
3filgrastim44 1210.7LIF, CSF1
4orcein449.6ACTC1, S100B
5mucicarmine449.5ACTC1, S100B
6ecori449.4MYB, S100B
7gm-csf44 2910.4CSF1, CSF1R, LIF
8bryostatin 1449.4CSF1, CSF1R
9adenine44 25 1211.4SLC29A3, MYB, S100B
10s 100449.4ACTC1, S100B, F13A1
11reticulin449.4ACTC1, F13A1, S100B
12b 723449.4S100B, ACTC1, F13A1
13sodium nitroprusside449.4CSF1, MYB, S100B
14hematoxylin449.4S100B, ACTC1, F13A1
151,25 dihydroxy vitamin d3449.3CSF1, LIF, CSF1R
16cytarabine44 50 1211.3CSF1R, LIF, CSF1
17methylcellulose449.3CSF1, MYB, LIF
18nitroblue tetrazolium449.2CSF1R, MYB, LIF
19phorbol449.2CSF1R, MYB, CSF1
20choline44 25 1211.2LIF, MYB, S100B
21agar449.1CSF1, LIF, CSF1R
22heparin44 29 25 1212.1F13A1, LIF, S100B, CSF1
23chloramphenicol44 3 50 1212.1S100B, MYB, CSF1
24dmso449.1CSF1, MYB, CSF1R
25wortmannin449.0LIF, CSF1, S100B, CSF1R
26thymidine44 259.8S100B, CSF1, MYB, LIF
27methionine448.8CSF1R, S100B, MYB, LIF
2812-o-tetradecanoylphorbol 13-acetate448.8MYB, LIF, CSF1R, CSF1
29iron44 259.7F13A1, ACTC1, S100B, MYB
30oligonucleotide448.7CSF1R, LIF, F13A1, MYB
31matrigel448.7LIF, CSF1, ACTC1
32glycogen44 259.7LIF, ACTC1, CSF1, S100B
33cycloheximide448.7CSF1, MYB, LIF, CSF1R
34creatinine448.6CSF1, S100B, LIF, ACTC1
35testosterone44 60 25 1211.5ACTC1, LIF, S100B, CSF1
36cyclosporin a44 29 6010.5ACTC1, S100B, LIF, MYB
37lipid448.3MYB, LIF, CSF1R, S100B, CD1A
38cysteine448.3CSF1, LIF, CSF1R, S100B, MYB
39arginine448.2S100B, MYB, LIF, CSF1
40imatinib44 50 1210.1ACTC1, S100B, CSF1, CSF1R, MYB
41serine448.0LIF, S100B, CSF1R, CSF1, MYB
42steroid448.0S100B, ACTC1, MYB, LIF, CSF1R
43paraffin447.9CSF1, ACTC1, CSF1R, S100B, F13A1, LIF
44calcium44 50 25 1210.8CD207, S100B, LIF, MYB, F13A1, ACTC1
45dexamethasone44 50 29 1210.6CSF1, CSF1R, MYB, S100B, LIF, ACTC1
46retinoic acid44 258.6MYB, LIF, ACTC1, CSF1, S100B, CSF1R
47tyrosine447.5LIF, S100B, CSF1, CSF1R, ACTC1, MYB
48progesterone44 29 60 25 1211.3F13A1, CSF1R, S100B, ACTC1, MYB, CSF1
49vegf447.3F13A1, LIF, MYB, ACTC1, CSF1R, CSF1
50estrogen447.3MYB, CSF1R, F13A1, S100B, CSF1, LIF

GO Terms for genes affiliated with Histiocytosis

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Cellular components related to Histiocytosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056158.3S100B, CSF1, ACTC1, LIF

Biological processes related to Histiocytosis according to GeneCards/GeneDecks:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of macrophage differentiationGO:0456519.6CSF1, LIF
2macrophage differentiationGO:0302259.6CSF1, CSF1R
3osteoclast differentiationGO:0303169.5CSF1R, CSF1
4hemopoiesisGO:0300979.5CSF1, CSF1R
5positive regulation of tyrosine phosphorylation of Stat3 proteinGO:0425179.3CSF1R, LIF
6cell proliferationGO:0082838.8S100B, CSF1, CSF1R
7positive regulation of cell proliferationGO:0082848.7S100B, CSF1, CSF1R, LIF

Products for genes affiliated with Histiocytosis

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Sources for Histiocytosis

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30KEGG
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57SNOMED-CT via Orphanet
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