MCID: HST010
MIFTS: 71

Histiocytosis malady

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

Summaries for Histiocytosis

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8Disease Ontology, 63Wikipedia, 32MalaCards
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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 etoposide and vinblastine 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

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8Disease Ontology, 10DISEASES, 44Novoseek, 60UMLS, 42NIH Rare Diseases, 48Orphanet, 39NCIt, 34MeSH, 56SNOMED-CT, 57SNOMED-CT via Orphanet, 26ICD10 via Orphanet
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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

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17GeneCards, 18GeneDecks
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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 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

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48Orphanet
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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

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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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

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Anatomical Context for Histiocytosis

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32MalaCards
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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

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36MGI
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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

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50PubMed
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Articles related to Histiocytosis:

(show top 50)    (show all 1328)
idTitleAuthorsYear
1
The genetics of interdigitating dendritic cell sarcoma share some changes with Langerhans cell histiocytosis in select cases. (24321462)
2014
2
Crystal-Storing Histiocytosis Masquerading Ocular Adnexal Lymphoma: A Case Report and Review of Literature. (24131949)
2013
3
Clinical Outcomes of Radiation Therapy in the Management of Langerhans Cell Histiocytosis. (23466581)
2013
4
Atypical histiocytosis in the lung. (23991320)
2013
5
Clofarabine salvage therapy for refractory high-risk langerhans cell histiocytosis. (23255383)
2013
6
Langerhans cell histiocytosis mimicking osteomyelitis in an infant. (24040605)
2013
7
Crystal-storing histiocytosis complicating primary pulmonary marginal zone lymphoma of mucosa-associated lymphoid tissue. (23991730)
2013
8
Saving orphans: BRAF targeting of histiocytosis. (23449613)
2013
9
Crystal-storing histiocytosis: a rare lesion in periapical pathology. (21849254)
2012
10
Langerhans cell histiocytosis of long bones: MR imaging and complete follow up study. (22842377)
2012
11
Measuring diffuse metabolic activity on FDG-PET/CT: new method for evaluating Langerhans cell histiocytosis activity in pulmonary parenchyma. (22172385)
2012
12
Notch is active in Langerhans cell histiocytosis and confers pathognomonic features on dendritic cells. (23074278)
2012
13
Pulmonary Langerhans cell histiocytosis and eosinophilic granuloma. (22880529)
2012
14
Langerhans cell histiocytosis - a mimicker of tuberculosis of the spine. (23217046)
2012
15
Intestinal lymphangiectasia associated with recurrence of histiocytosis X. (21947159)
2011
16
Quantitative PCR and immunohistochemical analyses of HMGB1 and RAGE expression in canine disseminated histiocytic sarcoma (malignant histiocytosis). (21617208)
2011
17
Residents' corner September 2011. (Carpe)DIEM - Birbeck granule in Langerhans cell histiocytosis. (21926046)
2011
18
Molluscumlike papules in a 4-month-old boy--quiz case. Langerhans cell histiocytosis (LCH)a89congenital self-healing reticulohistiocytosis. (21422345)
2011
19
A case of Langerhans cell histiocytosis in an adult with initial symptoms confined to the skin. (20465678)
2010
20
Successful treatment with cyclophosphamide of Langerhans cell histiocytosis involving the skin and lymph nodes in an adult patient. (20345994)
2010
21
Erythematous patch overlying a swollen knee--quiz case. Intralymphatic histiocytosis. (20855708)
2010
22
Langerhans cell histiocytosis causing cervical myelopathy in a child. (20617093)
2010
23
A case of a Korean adult affected by type B Niemann-Pick disease: secondary sea-blue histiocytosis and molecular characterization]. (19411774)
2009
24
Niemann-Pick disease, type B with TRAP-positive storage cells and secondary sea blue histiocytosis. (19864213)
2009
25
Langerhans cell histiocytosis masquerading as tuberculosis: a diagnostic dilemma resulting in inappropriate anti-tubercular therapy. (19148950)
2009
26
An 'eruptive' variant of juvenile xanthogranuloma associated with langerhans cell histiocytosis. (18840155)
2008
27
Increased blood myeloid dendritic cells and dendritic cell-poietins in Langerhans cell histiocytosis. (15728521)
2005
28
Sinus histiocytosis with massive lymphadenopathy: complicated by autoimmune hemolytic anemia. (14620195)
2003
29
Long-lasting "christmas tree rash" in an adolescent: isotopic response of indeterminate cell histiocytosis in pityriasis rosea? (12361135)
2002
30
Progressive nodular histiocytosis in a child with a hypothalamic tumor. (11852916)
2002
31
CD79a(+) T-cell lymphoblastic lymphoma with coexisting Langerhans cell histiocytosis. (11419987)
2001
32
The digestive system manifestations of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy): review of 11 cases. (10746683)
2000
33
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease): a rare cause of bilateral renal masses. (10587134)
1999
34
Genetic and immunological parameters governing in vivo susceptibility/resistance to retrovirally induced murine malignant histiocytosis. (9766624)
1998
35
Malignant histiocytosis in three Bernese mountain dogs. (8735262)
1996
36
Modulation of fibroblast activity in histiocytosis X by platelet-derived growth factor. (7874940)
1995
37
A unique non-Langerhans cell histiocytosis with some features of generalized eruptive histiocytoma. (8034797)
1994
38
Hematophagic histiocytosis: a clinicopathologic analysis of 23 cases with special reference to the association with peripheral T-cell lymphoma. (7920083)
1994
39
Epstein-Barr virus-containing T-cell lymphoma presents with hemophagocytic syndrome mimicking malignant histiocytosis. (8395969)
1993
40
Langerhans' cell histiocytosis presenting with the superior vena cava syndrome: a case report. (8515729)
1993
41
Benign cephalic histiocytosis: report of four cases. (2508072)
1989
42
B cell lymphoma showing clinicopathological features of malignant histiocytosis. (3124481)
1988
43
Maturation of Howell-Jolly bodies observed in a case of malignant histiocytosis. (3421016)
1988
44
Immature phenotypic conversion in a patient with malignant histiocytosis. (3766080)
1986
45
Fatal disseminated adenovirus infection featuring liver necrosis and prolonged viremia in an immunosuppressed child with malignant histiocytosis. (2994509)
1985
46
Malignant histiocytosis: case report and review. (3977614)
1985
47
Benign cephalic histiocytosis. A case report and ultrastructural study. (3973127)
1985
48
Malignant Histiocytosis. A clinicopathological study of 12 cases. (7166343)
1982
49
Oncornavirus-like particles from cultured bone marrow cells preceding leukemia and malignant histiocytosis. (52158)
1975
50
Acute disseminated histiocytosis X: a case with transition from eosinophilic granuloma of bone to Letterer-Siwe disease. (5793702)
1969

Genetic Variations for Histiocytosis

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Expression for genes affiliated with Histiocytosis

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Histiocytosis

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Pathways for genes affiliated with Histiocytosis

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29KEGG, 51QIAGEN
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Compounds for genes affiliated with Histiocytosis

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44Novoseek, 59Tocris Bioscience, 28IUPHAR, 11DrugBank, 24HMDB, 49PharmGKB, 2BitterDB
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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

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16Gene Ontology
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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

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  • Antibodies
  • Proteins
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Sources for Histiocytosis

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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