MCID: HST010
MIFTS: 71

Histiocytosis malady

Respiratory diseases, Bone diseases, Blood diseases, Cancer diseases categories

Summaries for Histiocytosis

About this section
Sources:
8Disease Ontology, 63Wikipedia, 32MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
Wikipedia:63 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 langerhans-cell histiocytosis and malignant histiocytosis, and has symptoms including diabetes insipidus, follicular/erythematous/edematous papules/milium and lung/pulmonary infiltrates. 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 thalidomide and etoposide and the compounds estrogen and progesterone have been mentioned in the context of this disorder. Affiliated tissues include bone, bone marrow and t cells, and 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

About this section
Sources:
8Disease Ontology, 10DISEASES, 44Novoseek, 60UMLS, 42NIH Rare Diseases, 48Orphanet, 39NCIt, 34MeSH, 56SNOMED-CT, 57SNOMED-CT via Orphanet, 26ICD10 via Orphanet
See all sources

Classifications:

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


Aliases & Descriptions:

histiocytosis 8 10 44 60
chronic and multifocal langerhans cell histiocytosis 48
multifocal eosinophilic granuloma 48
hand-schuller-christian syndrome 42
hand-schüller-christian disease 48
hand-schuller-christian disease 42
hand schuller christian disease 8
histiocytosis, langerhans-cell 60
chronic histiocytosis x 8


External Ids:

Disease Ontology8 DOID:3405
NCIt39 C3106
MeSH34 D015614
SNOMED-CT via Orphanet57 39795003
ICD10 via Orphanet26 C96.5

Related Diseases for Histiocytosis

About this section
Sources:
17GeneCards, 18GeneDecks
See all sources

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 361)
idRelated DiseaseScoreTop Affiliating Genes
1langerhans-cell histiocytosis31.8S100A1, F13A1, S100B
2malignant histiocytosis31.5CSF1, TNFRSF8, SERPINA3, MYB, CSF1R
3leukemia30.8SERPINA3, CSF1R, CSF1, CTSD, CD1A, MYB
4juvenile xanthogranuloma30.6F13A1, S100B, FSCN1
5acute leukemia30.6CSF1R, CSF1, MYB, RARA
6letterer-siwe disease30.5RARA, CSF1, S100B, FSCN1, CD1A, CD40
7sarcoma30.5ACTC1, IL1R1, FSCN1, S100B, S100A1, CSF1R
8langerhans cell sarcoma30.3CD207
9hodgkin's lymphoma30.3FSCN1, CSF1R, TNFRSF8, CD68, CD40
10arthritis30.1SERPINA3, CSF1, CSF1R, IL1R1, CD163, LIF
11monocytic leukemia30.1CD40, CSF1, LIF, RARA, IL1R1
12rheumatoid arthritis30.1IL1R1, SERPINA3, CSF1, CD40, CD68, CD163
13anaplastic large cell lymphoma30.1CSF1R, FSCN1, TNFRSF8
14myeloid leukemia30.1RARA, LIF, CSF1R, CSF1, MYB
15pneumonia30.1CD40
16myeloma30.1CD40, TNFRSF8, LIF, CSF1, RARA
17acute monocytic leukemia30.1RARA, CSF1, CD40
18skin disease30.1CD68
19fibrous histiocytoma30.0S100B, F13A1, SERPINA3, ACTC1, CD163, FSCN1
20malaria30.0LIF, SERPINA3
21amyloidosis30.0SERPINA3, CTSD
22sarcoidosis30.0SERPINA3
23dermatitis30.0LIF, F13A1
24non-hodgkin lymphoma30.0TNFRSF8, CD40, MYB, FSCN1
25meningioma29.8S100A1, CD68, CTSD, S100B
26adenocarcinoma29.8SERPINA3, RARA, F13A1, LIF, MYB, FSCN1
27inflammatory bowel disease29.8CD40, IL1R1, SERPINA3, F13A1
28idiopathic pulmonary fibrosis29.8ACTC1, IL1R1, S100A1
29lung cancer29.8CSF1R, CD40, RARA, FSCN1
30diabetes mellitus29.8IL1R1
31rosai-dorfman disease29.6S100B, CTSD, CD163, FSCN1
32asthma29.6SERPINA3, TNFRSF8, CD1A, CSF1, LIF
33osteoarthritis29.6S100A1, CSF1R, CSF1, F13A1, LIF, CD68
34papilloma29.6FSCN1, ACTC1, RARA
35astrocytoma29.6FSCN1, RARA, S100B, SERPINA3
36glioblastoma multiforme29.6S100B, RARA, CSF1R, MYB, CTSD
37neutropenia29.6CSF1, LIF, CD40
38squamous cell carcinoma29.6S100A1, ACTC1, FSCN1, RARA, CTSD, SERPINA3
39stomach cancer29.6RARA, IL1R1, SERPINA3, LIF, CTSD, FSCN1
40severe combined immunodeficiency29.6CD40, CSF1, TNFRSF8
41multicentric reticulohistiocytosis29.6CD68
42obesity29.6LIF, RARA, CTSD
43spindle cell sarcoma29.6ACTC1, S100B
44basal cell carcinoma29.6ACTC1, RARA, F13A1
45disseminated intravascular coagulation29.6RARA, LIF
46aspergillosis29.6CSF1
47thyroid cancer29.6CTSD, S100A1, RARA
48graves' disease29.6CD40
49cholesteatoma29.6S100A1, RARA
50non-langerhans-cell histiocytosis10.5

Graphical network of the top 20 diseases related to Histiocytosis:



Diseases related to histiocytosis

Clinical Features for Histiocytosis

About this section
Sources:
48Orphanet
See all sources


Symptoms:

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

Drugs & Therapeutics for Histiocytosis

About this section
Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-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 UMLS60/NDF-RT40:

Genetic Tests for Histiocytosis

About this section

Anatomical Context for Histiocytosis

About this section
Sources:
32MalaCards
See all sources

MalaCards organs/tissues related to Histiocytosis:

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

Animal Models for Histiocytosis or affiliated genes

About this section
Sources:
36MGI
See all sources

MGI Mouse Phenotypes related to Histiocytosis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000539710.5SLC29A3, CSF1R, CSF1, TNFRSF8, CTSD, F13A1
2MP:000539010.5CSF1, LIF, CD68, CD40, RARA, CSF1R
3MP:000538710.4LIF, CTSD, TNFRSF8, CSF1, CSF1R, MYB
4MP:000538410.3CSF1R, CSF1, CTSD, LIF, MYB, CD40
5MP:000538510.2S100A1, CSF1, F13A1, LIF, MYB, IL1R1

Publications for Histiocytosis

About this section
Sources:
50PubMed
See all sources

Articles related to Histiocytosis:

(show top 50)    (show all 1328)
idTitleAuthorsYear
1
Soft tissue Langerhans cell histiocytosis with secondary bone involvement in extremities: evolution of lesions in two patients. (23609170)
2013
2
The adverse prognostic hallmarks in identical twins with Langerhans cell histiocytosis: a clinical report and literature review. (23924960)
2013
3
Langerhans cell histiocytosis associated with lichen sclerosus of the vulva: case report and review of the literature. (23167438)
2013
4
Spontaneous remission of 2-chlorodeoxyadenosine (2-CdA)-related secondary myelodysplastic syndrome in a patient with refractory Langerhans cell histiocytosis. (23616222)
2013
5
Cutaneous Langerhans cell histiocytosis: study of Asian children shows good overall prognosis. (23909818)
2013
6
Langerhans cell histiocytosis of the urinary bladder in a patient with bladder cancer previously treated with intravesical Bacillus Calmette-GuAcrin therapy. (24332601)
2013
7
"Blueberry muffin" rash and large right thigh mass: a unique presentation of Langerhans cell histiocytosis. (24011317)
2013
8
Pathogenesis of Langerhans cell histiocytosis. (22906202)
2013
9
Benign cephalic histiocytosis. (23974593)
2013
10
Lenalidomide proved effective in multisystem Langerhans cell histiocytosis. (22103591)
2012
11
Multifocal Langerhans cell histiocytosis in an adult. (22214636)
2012
12
Multisystemic Langerhans cell histiocytosis with advanced lung involvement. (23372865)
2012
13
Indeterminate cell histiocytosis in a pediatric patient: successful treatment with thalidomide. (21688087)
2012
14
Paediatric spinal Langerhans cell histiocytosis requiring corpectomy and fusion at C7 and at Th8-Th9 levels. (23264157)
2012
15
Langerhans cell histiocytosis: pragmatic empirism on the road to rational cure. (22712720)
2012
16
Clinical pathologic conference case 2: Langerhans cell histiocytosis. (21874507)
2011
17
A rare case of "switch on and off" multi-system Langerhans cell histiocytosis in an adult patient. (21745366)
2011
18
Classical Hodgkin's lymphoma associated with Langerhans cell histiocytosis: multiagent chemotherapy resulted in histologic resolution of both the classical Hodgkin's lymphoma and Langerhans cell proliferation components. (21041711)
2011
19
Pulmonary langerhans cell histiocytosis masquerading as tuberculosis in an infant. (22041471)
2011
20
Langerhans' cell histiocytosis of the liver in adults. (21550330)
2011
21
Langerhans cell histiocytosis with thyroid and lung involvement in a child: a case report. (20224443)
2010
22
Multifocal Langerhans cell histiocytosis with infiltrative pelvic lesions: PET/CT imaging. (20838300)
2010
23
Indeterminate cell histiocytosis in association with acute myeloid leukemia. (20672000)
2010
24
Langerhans cell histiocytosis: a case study suggesting a reactive aetiology. (20302571)
2010
25
Immunocytochemical investigation of Langerin (CD207) is a valuable adjunct in the cytological diagnosis of Langerhans cell histiocytosis of the thyroid. (19195798)
2009
26
Sonography of biopsy-proven Langerhans cell histiocytosis in lymph nodes of the neck. (19321680)
2009
27
Concurrent Langerhans cell histiocytosis and nephroblastoma. (19148948)
2009
28
Non-Langerhans cell histiocytosis in a child with Kawasaki disease. (21686403)
2009
29
A rare histiocytosis with severe cardiac involvement: Erdheim-Chester disease. (18717219)
2008
30
Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha. (16020507)
2005
31
Serum levels of interleukin-1 receptor antagonist and tumor necrosis factor-alpha are elevated in children with Langerhans cell histiocytosis. (12794527)
2003
32
Ocular involvement in a case of systemic indeterminate cell histiocytosis: a case report. (11588435)
2001
33
Langerhans cell histiocytosis of an adult with tumors in liver and spleen. (11379359)
2001
34
An Asian variant of intravascular lymphomatosis: an updated review of malignant histiocytosis-like B-cell lymphoma. (10342574)
1999
35
Primary malignant histiocytosis of the brain in a dog. (10373296)
1999
36
True malignant histiocytosis developed during chemotherapy for mediastinal immature teratoma. (8892599)
1996
37
Successful treatment of two children with Langerhans' cell histiocytosis with 2'-deoxycoformycin. (8846128)
1996
38
Bone marrow findings in malignant histiocytosis and/or malignant lymphoma with concurrent hemophagocytic syndrome. (8161938)
1993
39
Sea blue histiocytosis: a common abnormality of the bone marrow in myelodysplastic syndromes. (8254090)
1993
40
Clinicopathological diagnosis and treatment of malignant histiocytosis. (2207002)
1990
41
Expression of natural killer (NK) antigens in malignant histiocytosis and a subset of acute myelomonocytic leukaemias. (2627603)
1989
42
Langerhans cell histiocytosis with the radiographic findings of Erdheim-Chester disease. (3258103)
1988
43
Radiotherapy in benign orbital disease. II: Ophthalmic Graves' disease and orbital histiocytosis X. (3259894)
1988
44
Malignant histiocytosis with clinical presentation of compression of cauda equina. (3251971)
1988
45
Monoclonal antibodies against SU-DHL-1 cells stain the neoplastic cells in true histiocytic lymphoma, malignant histiocytosis, and Hodgkin's disease. (2424524)
1986
46
Virus-associated hemophagocytic syndrome or malignant histiocytosis? (4067762)
1985
47
Erythema annulare centrifugum and malignant histiocytosis--report of a case. (6499278)
1984
48
Malignant histiocytosis and ulcerative jejunitis of the small intestine. (6839654)
1983
49
Acute lymphocytic leukaemia terminating in malignant histiocytosis. A case report and literature review. (6929112)
1980
50
Malignant histiocytosis (histiocytic medullary reticulosis) with spindle cell differentiation and tumour formation. (845260)
1977

Genetic Variations for Histiocytosis

About this section

Expression for genes affiliated with Histiocytosis

About this section
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

About this section
Sources:
29KEGG, 51QIAGEN
See all sources

Compounds for genes affiliated with Histiocytosis

About this section
Sources:
44Novoseek, 59Tocris Bioscience, 28IUPHAR, 11DrugBank, 24HMDB, 49PharmGKB, 2BitterDB
See all sources

Compounds related to Histiocytosis according to GeneCards/GeneDecks:

(show top 50)    (show all 90)
idCompoundScoreTop Affiliating Genes
1estrogen4410.9F13A1
2progesterone44 59 28 11 2414.9CD40, CSF1R, IL1R1, RARA, ACTC1, SERPINA3
3vegf4410.9IL1R1, ACTC1, LIF, F13A1, CTSD, CSF1
4dexamethasone44 49 28 1113.9S100B, CSF1R, MYB, CD163, IL1R1, RARA
512-o-tetradecanoylphorbol 13-acetate4410.9CD40, IL1R1, FSCN1, RARA, CSF1, LIF
6retinoic acid44 2411.9CSF1R, CD40, MYB, LIF, CTSD, CSF1
7steroid4410.9S100B, SERPINA3, ACTC1, RARA, CD40, IL1R1
8creatinine4410.9CSF1, CTSD, SERPINA3, ACTC1
9methionine4410.9CSF1R, LIF, MYB, IL1R1, S100B, SERPINA3
10thymidine44 2411.9CTSD, TNFRSF8, S100B, CSF1, LIF, MYB
11cyclosporin a44 28 5912.9ACTC1, S100B, TNFRSF8, CTSD, LIF, MYB
12iron44 2411.9S100B, SERPINA3, S100A1, F13A1, MYB, CD163
13serine4410.9S100B, CSF1R, CSF1, LIF, IL1R1, SERPINA3
14cysteine4410.9S100A1, CSF1R, CSF1, RARA, MYB, LIF
15cycloheximide4410.9CSF1R
16imatinib44 49 1112.9RARA, MYB, ACTC1, CSF1, S100B, CSF1R
17lipid4410.9TNFRSF8, LIF, CSF1R, MYB, S100B, CD68
18calcium44 49 11 2413.9TNFRSF8, ACTC1, FSCN1, LIF, F13A1, CTSD
19paraffin4410.9CSF1, CTSD, F13A1, LIF, CD163, CD68
20oxygen44 2411.9CD40, SERPINA3, S100B, MYB, CTSD
21b 7234410.9S100B, F13A1, S100A1, ACTC1
22nitroblue tetrazolium4410.9LIF, RARA, CSF1R, MYB
23p0034410.8CSF1, CTSD, S100B
24tyrosine4410.8S100A1, S100B, IL1R1, CSF1R, CSF1, CD40
25oligonucleotide4410.8LIF, SERPINA3, RARA, CSF1R, F13A1, MYB
26zinc44 2411.8S100A1, TNFRSF8, CTSD, S100B, MYB, CD40
27heparin44 28 11 2413.8CSF1, S100B, SERPINA3, IL1R1, F13A1, LIF
28phorbol4410.8MYB, CSF1R, IL1R1, CD40, CSF1
29etoposide44 49 59 1113.8RARA, IL1R1, CTSD, CSF1, CSF1R, S100B
30s 1004410.8ACTC1, F13A1, S100B
31paclitaxel44 49 1112.8CTSD, LIF, CSF1, RARA, S100B
32il 104410.8IL1R1, CD40, LIF, CSF1
33threonine4410.8CD40, IL1R1, FSCN1, TNFRSF8, CSF1R, S100B
34glycogen44 2411.8ACTC1, LIF, CSF1
35mannose4410.8CD163, CD207, CD40, CTSD, CSF1
36actinomycin d4410.8RARA, LIF, MYB, CD40, CSF1, IL1R1
37testosterone44 59 11 2413.7S100B, ACTC1, RARA, IL1R1, LIF, CTSD
38hematoxylin4410.7S100B, ACTC1, F13A1, S100A1
39estradiol44 11 2412.7RARA, CTSD, MYB
40reticulin4410.7ACTC1
41mucicarmine4410.7S100A1, ACTC1, S100B
42chloramphenicol44 2 1112.7CD40, MYB, CSF1, S100B, RARA
43agar4410.6LIF, RARA, CSF1, CTSD, CSF1R
44silver4410.6CTSD, S100A1, F13A1, SERPINA3
45arginine4410.6S100B, SERPINA3, CSF1, RARA, IL1R1, MYB
46phosphatidylinositol4410.5CD40, RARA, CSF1R, IL1R1, CSF1, CTSD
47ascorbic acid44 2411.5S100B, CSF1, IL1R1, LIF, CTSD
48nitric oxide44 11 2412.4S100B, LIF, CD40, S100A1, CTSD, RARA
49ribonucleic acid4410.4LIF, IL1R1, CD40, ACTC1, RARA
50alanine4410.2CTSD, MYB, S100B, IL1R1, CSF1, RARA

GO Terms for genes affiliated with Histiocytosis

About this section
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:00828410.5RARA, LIF, CSF1, CSF1R, S100B
2macrophage differentiationGO:03022510.5CSF1, CSF1R
3positive regulation of tyrosine phosphorylation of Stat1 proteinGO:04251110.5CD40, LIF
4positive regulation of macrophage differentiationGO:04565110.4CSF1, LIF
5inflammatory responseGO:00695410.2SERPINA3, CD40, CSF1, CSF1R

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

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Histiocytosis

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet