MCID: HST010
MIFTS: 61

Histiocytosis malady

Respiratory, Blood, Cancer categories

Summaries for Histiocytosis

Sources:
8Disease Ontology, 64Wikipedia, 33MalaCards
See all sources

Fully expand this MalaCard

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

MalaCards: Histiocytosis, also known as chronic and multifocal langerhans cell histiocytosis, is related to malignant histiocytosis and rosai-dorfman disease, and has symptoms including proptosis/exophthalmos, diabetes insipidus and osteolysis/osteoclasia/bone destruction/erosions. An important gene associated with Histiocytosis is F13A1 (coagulation factor XIII, A1 polypeptide), and among its related pathways are Hematopoietic cell lineage and PAK Pathway. The drugs etoposide and vinblastine and the compounds estrogen and progesterone have been mentioned in the context of this disorder. Related mouse phenotypes are hematopoietic system and skeleton.

Disease Ontology:8 A reticuloendotheliosis that is characterized by an excessive number of histiocytes.

Aliases & Classifications for Histiocytosis

Sources:
8Disease Ontology, 10DISEASES, 45Novoseek, 61UMLS, 43NIH Rare Diseases, 49Orphanet, 40NCIt, 35MeSH, 57SNOMED-CT, 26ICD10 via Orphanet
See all sources

Classifications:

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


Aliases & Descriptions:

histiocytosis 8 10 45 61
chronic and multifocal langerhans cell histiocytosis 49
multifocal eosinophilic granuloma 49
hand-schuller-christian syndrome 43
hand-schüller-christian disease 49
hand-schuller-christian disease 43
hand schuller christian disease 8
histiocytosis, langerhans-cell 61
chronic histiocytosis x 8


External Ids:

Disease Ontology8 DOID:3405
NCIt40 C3106
MeSH35 D015614
ICD10 via Orphanet26 C96.5

Related Diseases for Histiocytosis

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Histiocytosis family:

malignant histiocytosis histiocytosis with joint contractures and sensorineural deafness

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

(show top 50)    (show all 343)
idRelated DiseaseScoreTop Affiliating Genes
1malignant histiocytosis31.6SERPINA3, MYB, TNFRSF8, CSF1, CSF1R
2rosai-dorfman disease31.0FSCN1, CD163, CTSD, S100B
3juvenile xanthogranuloma30.7S100B, F13A1, FSCN1
4acute leukemia30.7CSF1R, CSF1, MYB, RARA
5letterer-siwe disease30.6LIF, F13A1, CTSD, CSF1, S100B, CD68
6langerhans cell sarcoma30.3CD207
7arthritis30.2CSF1R, CSF1, LIF, CD163, IL1R1, SERPINA3
8anaplastic large cell lymphoma30.2CSF1R, TNFRSF8, FSCN1
9acute monocytic leukemia30.1CSF1, CD40, RARA
10fibrous histiocytoma30.0S100B, F13A1, CD163, FSCN1, ACTC1, SERPINA3
11amyloidosis30.0SERPINA3, CTSD
12adenocarcinoma29.9CSF1R, CTSD, F13A1, LIF, MYB, FSCN1
13teratoma29.9LIF, ACTC1
14idiopathic pulmonary fibrosis29.9ACTC1, IL1R1, S100A1
15asthma29.7CSF1, TNFRSF8, LIF, CD1A, SERPINA3
16osteoarthritis29.7S100A1, CSF1R, CSF1, F13A1, LIF, CD68
17myelodysplastic syndromes29.7RARA, CSF1, CSF1R
18astrocytoma29.7S100B, FSCN1, RARA, SERPINA3
19fibromatosis29.7ACTC1, S100A1
20squamous cell carcinoma29.7S100A1, CSF1R, CTSD, LIF, MYB, IL1R1
21stomach cancer29.7CTSD, LIF, IL1R1, FSCN1, RARA, SERPINA3
22spindle cell sarcoma29.7S100B, ACTC1
23acute myelomonocytic leukemia29.7CSF1, CSF1R
24anaplastic astrocytoma29.7FSCN1
25basal cell carcinoma29.7F13A1, RARA, ACTC1
26aspergillosis29.7CSF1
27thyroid cancer29.7RARA, CTSD, S100A1
28graves' disease29.7CD40
29benign cephalic histiocytosis10.6
30indeterminate cell histiocytosis10.5
31erdheim-chester disease10.5
32diabetes insipidus10.5
33generalized eruptive histiocytosis10.4
34progressive nodular histiocytosis10.4
35adult pulmonary langerhans cell histiocytosis10.3
36hereditary progressive mucinous histiocytosis10.3
37niemann-pick disease10.3
38niemann–pick disease10.3
39histiocytosis-lymphadenopathy plus syndrome10.2
40gaucher's disease10.2
41sea-blue histiocytosis10.2
42histiocytic sarcoma10.2
43pick's disease10.2
44t-cell leukemia10.2
45alk+ histiocytosis10.2
46acute t cell leukemia10.1
47n syndrome10.1
48histiocytosis with joint contractures and sensorineural deafness10.1
49lymphoblastic lymphoma10.1
50lymphomatoid papulosis10.1

Graphical network of the top 20 diseases related to Histiocytosis:



Diseases related to histiocytosis

Clinical Features for Histiocytosis

Sources:
49Orphanet
See all sources


Symptoms:

49 (show all 7)
  • proptosis/exophthalmos
  • diabetes insipidus
  • osteolysis/osteoclasia/bone destruction/erosions
  • follicular/erythematous/edematous papules/milium
  • hepatomegaly/liver enlargement (excluding storage disease)
  • lung/pulmonary infiltrates
  • lymphadenopathy/polyadenopathies

Drugs & Therapeutics for Histiocytosis

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

Approved drugs:

Search CenterWatch for Histiocytosis

Drug clinical trials:

Search ClinicalTrials for Histiocytosis

Search NIH Clinical Center for Histiocytosis

Search CenterWatch for Histiocytosis

Inferred drug relations via UMLS61/NDF-RT41:

Genetic Tests for Histiocytosis

Anatomical Context for Histiocytosis

Animal Models for Histiocytosis or affiliated genes

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

Publications for Histiocytosis

Sources:
51PubMed
See all sources

Articles related to Histiocytosis:

(show top 50)    (show all 1335)
idTitleAuthorsYear
1
Rapid resolution of pulmonary Langerhans cell histiocytosis. (23749831)
2013
2
Identification of the V600D mutation in Exon 15 of the BRAF oncogene in congenital, benign langerhans cell histiocytosis. (22996177)
2013
3
Recurrent Langerhans cell histiocytosis of the mandible. (23131794)
2013
4
Sonographic diagnosis and monitoring of localized langerhans cell histiocytosis of the skull. (22987564)
2013
5
Intralesional infiltration of corticosteroids in the treatment of localized Langerhans cell histiocytosis of the mandible: report of two cases. (22771407)
2013
6
Langerhans cell histiocytosis of the temporal bone. (21970849)
2012
7
Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy. (22835048)
2012
8
Crystal-storing histiocytosis: report of a case, review of the literature (80 cases) and a proposed classification. (22430767)
2012
9
Recent advances in the understanding of Langerhans cell histiocytosis. (22017623)
2012
10
Cladribine is effective against cystic pulmonary Langerhans cell histiocytosis. (23118088)
2012
11
Crystal storing histiocytosis of the tongue as the initial presentation of multiple myeloma. (21420638)
2011
12
Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study. (22151964)
2011
13
Cytomorphology of crystal storing histiocytosis in the breast associated with lymphoma: a case report. (21525745)
2011
14
Simultaneous Occurrence of Hodgkin's Lymphoma and Langerhans Cell Histiocytosis of the Spine : A Rare Combination. (20461174)
2010
15
A child with coexistent juvenile xanthogranuloma and Langerhans cell histiocytosis. (19969387)
2010
16
Crystal-storing histiocytosis associated with only one of two consecutive, but genetically unrelated B-cell lymphomas. (19106020)
2009
17
Progressive diffuse pulmonary Langerhans cell histiocytosis improved by cladribine chemotherapy. (19252036)
2009
18
Case of the month #139. Central nervous system Langerhans cell histiocytosis. (19385155)
2008
19
Crystal-storing histiocytosis associated with MALT lymphoma of the ocular adnexa: a case report with review of literature. (17111127)
2007
20
Clinical spectrum of cutaneous Langerhans' cell histiocytosis mimicking various diseases. (16585988)
2006
21
Dramatic change of Ga-67 citrate uptake before and after corticosteroid therapy in a case of cardiac histiocytosis (Erdheim-Chester disease). (17174817)
2006
22
Benign cephalic histiocytosis in a British-African child. (16190998)
2005
23
Secondary hemophagocytic lymphohistiocytosis induced by malaria infection in a child with Langerhans cell histiocytosis. (15202166)
2004
24
Pathologic quiz case: persistent localized inguinal adenopathy in a man. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease). (12873185)
2003
25
Severe autoimmune neutropenia associated with bone marrow sea-blue histiocytosis. (12199768)
2002
26
Malignant histiocytosis in cattle. (11193337)
2000
27
Indeterminate-cell histiocytosis: immunophenotypic and cytogenetic findings in an infant. (10102017)
1999
28
Malignant histiocytosis in leukemia phase: Case report and review of the literature. (17372473)
1996
29
Malignant histiocytosis in childhood: a disease in quest of new nosological criteria. (7603402)
1995
30
Malignant histiocytosis derived from a common histiocyte clone in a patient with chronic Epstein-Barr virus infection. (8580809)
1995
31
Regressing atypical histiocytosis: report of two cases with progression to high grade T-cell non-Hodgkin's lymphoma. (8385660)
1993
32
Visceral leishmaniasis with a very-low degree of bone-marrow parasitemia, mimicking malignant histiocytosis. (21573388)
1993
33
Malignant histiocytosis in childhood: a distinctive CD30-positive clinicopathological entity associated with a chromosomal translocation involving 5q35. (1313990)
1992
34
Tumours classified as "malignant histiocytosis" in children are T-cell neoplasms. (1319178)
1992
35
Hyperphenylalaninemia in malignant histiocytosis and virus-associated hemophagocytic syndrome. (3389140)
1988
36
Lymphohistiocytosis in childhood. Pathologic comparison with fatal Letterer-Siwe disease (disseminated visceral histiocytosis X). (3822757)
1987
37
Mediastinal germ cell tumour associated with malignant histiocytosis and high rubella titres. (3770648)
1986
38
Malignant histiocytosis (histiocytic sarcoma). A (the?) major cause of the 'midline granuloma syndrome'. (3515840)
1986
39
Malignant histiocytosis in four dogs. (4055515)
1985
40
Malignant histiocytosis presenting with skin ulceration. (7175892)
1982
41
Intravascular malignant histiocytosis mimicking central nervous system vasculitis: an immunopathological diagnostic approach. (6758680)
1982
42
Respiratory failure in malignant histiocytosis. (7305121)
1981
43
A cytochemical and immunohistochemical approach to malignant histiocytosis. (6167344)
1981
44
Therapy of malignant histiocytosis. (7327431)
1981
45
Malignant histiocytosis showing ascites and recurrent meningeal infiltration. (6931467)
1980
46
Malignant histiocytosis simulating granulomatous disease. (7405896)
1980
47
Disseminated histiocytosis and intestinal malakoplakia. Occurrence due to Mycobacterium intracellulare infection. (582266)
1979
48
Malignant histiocytosis: a clinico-pathological study of 12 cases. (708640)
1978
49
Histiocytosis X? Necrobiosis lipoidica. (6019102)
1967
50
Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and SchA1ller-Christian disease as related manifestations of a single nosologic entity. (13057466)
1953

Genetic Variations for Histiocytosis

Expression for genes affiliated with Histiocytosis

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
30KEGG, 52QIAGEN
See all sources

Compounds for genes affiliated with Histiocytosis

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

Compounds related to Histiocytosis according to GeneCards/GeneDecks:

(show top 50)    (show all 90)
idCompoundScoreTop Affiliating Genes
1estrogen4510.9F13A1
2progesterone45 60 29 11 2414.9CD40, CSF1R, IL1R1, RARA, ACTC1, SERPINA3
3vegf4510.9IL1R1, ACTC1, LIF, F13A1, CTSD, CSF1
4dexamethasone45 50 29 1113.9S100B, CSF1R, MYB, CD163, IL1R1, RARA
512-o-tetradecanoylphorbol 13-acetate4510.9CD40, IL1R1, FSCN1, RARA, CSF1, LIF
6retinoic acid45 2411.9CSF1R, CD40, MYB, LIF, CTSD, CSF1
7steroid4510.9S100B, SERPINA3, ACTC1, RARA, CD40, IL1R1
8creatinine4510.9CSF1, CTSD, SERPINA3, ACTC1
9methionine4510.9CSF1R, LIF, MYB, IL1R1, S100B, SERPINA3
10thymidine45 2411.9CTSD, TNFRSF8, S100B, CSF1, LIF, MYB
11cyclosporin a45 29 6012.9ACTC1, S100B, TNFRSF8, CTSD, LIF, MYB
12iron45 2411.9S100B, SERPINA3, S100A1, F13A1, MYB, CD163
13serine4510.9S100B, CSF1R, CSF1, LIF, IL1R1, SERPINA3
14cysteine4510.9S100A1, CSF1R, CSF1, RARA, MYB, LIF
15cycloheximide4510.9CSF1R
16imatinib45 50 1112.9RARA, MYB, ACTC1, CSF1, S100B, CSF1R
17lipid4510.9TNFRSF8, LIF, CSF1R, MYB, S100B, CD68
18calcium45 50 11 2413.9TNFRSF8, ACTC1, FSCN1, LIF, F13A1, CTSD
19paraffin4510.9CSF1, CTSD, F13A1, LIF, CD163, CD68
20oxygen45 2411.9CD40, SERPINA3, S100B, MYB, CTSD
21b 7234510.9S100B, F13A1, S100A1, ACTC1
22nitroblue tetrazolium4510.9LIF, RARA, CSF1R, MYB
23p0034510.8CSF1, CTSD, S100B
24tyrosine4510.8S100A1, S100B, IL1R1, CSF1R, CSF1, CD40
25oligonucleotide4510.8LIF, SERPINA3, RARA, CSF1R, F13A1, MYB
26zinc45 2411.8S100A1, TNFRSF8, CTSD, S100B, MYB, CD40
27heparin45 29 11 2413.8CSF1, S100B, SERPINA3, IL1R1, F13A1, LIF
28phorbol4510.8MYB, CSF1R, IL1R1, CD40, CSF1
29etoposide45 50 60 1113.8RARA, IL1R1, CTSD, CSF1, CSF1R, S100B
30s 1004510.8ACTC1, F13A1, S100B
31paclitaxel45 50 1112.8CTSD, LIF, CSF1, RARA, S100B
32il 104510.8IL1R1, CD40, LIF, CSF1
33threonine4510.8CD40, IL1R1, FSCN1, TNFRSF8, CSF1R, S100B
34glycogen45 2411.8ACTC1, LIF, CSF1
35mannose4510.8CD163, CD207, CD40, CTSD, CSF1
36actinomycin d4510.8RARA, LIF, MYB, CD40, CSF1, IL1R1
37testosterone45 60 11 2413.7S100B, ACTC1, RARA, IL1R1, LIF, CTSD
38hematoxylin4510.7S100B, ACTC1, F13A1, S100A1
39estradiol45 11 2412.7RARA, CTSD, MYB
40reticulin4510.7ACTC1
41mucicarmine4510.7S100A1, ACTC1, S100B
42chloramphenicol45 2 1112.7CD40, MYB, CSF1, S100B, RARA
43agar4510.6LIF, RARA, CSF1, CTSD, CSF1R
44silver4510.6CTSD, S100A1, F13A1, SERPINA3
45arginine4510.6S100B, SERPINA3, CSF1, RARA, IL1R1, MYB
46phosphatidylinositol4510.5CD40, RARA, CSF1R, IL1R1, CSF1, CTSD
47ascorbic acid45 2411.5S100B, CSF1, IL1R1, LIF, CTSD
48nitric oxide45 11 2412.4S100B, LIF, CD40, S100A1, CTSD, RARA
49ribonucleic acid4510.4LIF, IL1R1, CD40, ACTC1, RARA
50alanine4510.2CTSD, MYB, S100B, IL1R1, CSF1, RARA

GO Terms for genes affiliated with Histiocytosis

Sources:
16Gene Ontology
See all sources

Cellular components related to Histiocytosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:00557610.5SERPINA3, IL1R1, CD40, CD163, F13A1, CTSD

Biological processes related to Histiocytosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of cell proliferationGO:00828410.5CSF1R, RARA, LIF, CSF1, S100B
2macrophage differentiationGO:03022510.5CSF1, CSF1R
3positive regulation of tyrosine phosphorylation of Stat1 proteinGO:04251110.5LIF, CD40
4positive regulation of macrophage differentiationGO:04565110.4LIF, CSF1
5inflammatory responseGO:00695410.2CSF1R, CSF1, CD40, SERPINA3

Molecular functions related to Histiocytosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1S100 protein bindingGO:04454810.5S100A1, S100B

Products for genes affiliated with Histiocytosis

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for 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