KH
MCID: KPS002
MIFTS: 40

Kaposiform Hemangioendothelioma (KH) malady

Rare diseases, Cardiovascular diseases, Fetal diseases, Cancer diseases categories

Summaries for Kaposiform Hemangioendothelioma

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MalaCards: Kaposiform Hemangioendothelioma, also known as congenital cutaneous multifocal kaposiform hemangioendothelioma, is related to hemangioendothelioma and hemangioma. An important gene associated with Kaposiform Hemangioendothelioma is FLT4 (fms-related tyrosine kinase 4), and among its related pathways is Lymphocyte Signaling. The compounds paraffin and vegf have been mentioned in the context of this disorder. Affiliated tissues include skin, endothelial and tonsil, and related mouse phenotypes are cardiovascular system and liver/biliary system.

Aliases & Classifications for Kaposiform Hemangioendothelioma

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44NIH Rare Diseases, 50Orphanet, 63UMLS, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet, 37MESH via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

50
kaposiform hemangioendothelioma:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

kaposiform hemangioendothelioma 44 50 63
congenital cutaneous multifocal kaposiform hemangioendothelioma 44
kaposiform hemangio-endothelioma 44
khe 44
kh 44


External Ids:

ICD10 via Orphanet27 D18.0
SNOMED-CT via Orphanet60 403983000
UMLS via Orphanet64 C1367420
MESH via Orphanet37 C537007

Related Diseases for Kaposiform Hemangioendothelioma

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18GeneCards, 19GeneDecks
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Diseases related to Kaposiform Hemangioendothelioma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show all 48)
idRelated DiseaseScoreTop Affiliating Genes
1hemangioendothelioma31.7FLI1, FLT4, CD34
2hemangioma30.5FLI1, CD34, PROX1, FLT4
3lymphedema30.4PROX1, FLT4
4lymphangioma30.3PROX1, FLT4, CD34
5sarcoma30.2FLI1, FLT4, CD34
6acute leukemia29.9FLT4, CD34
7leukemia29.9FLT4, FLI1, CD34
8tufted angioma10.6
9mediastinitis10.5
10lymphangiomatosis10.5
11endotheliitis10.4
12fragile x syndrome10.3
13tonsillitis10.2
14hereditary lymphedema10.2
15cervicitis10.2
16laryngitis10.2
17pericardial effusion10.2
18hemophilia10.2
19hemangioma thrombocytopenia syndrome10.2
20soft tissue sarcoma10.2
21lymphatic malformation10.2
22lymphangiosarcoma10.1PROX1
23kaposi's sarcoma10.0CD34
24synovial sarcoma10.0FLI1, CD34
25liposarcoma10.0FLI1, CD34
26neuronitis10.0
27presbyopia10.0
28muscular atrophy10.0
29macroglobulinemia10.0
30amyloidosis10.0
31brain stem infarction10.0
32inflammatory bowel disease10.0
33spinal muscular atrophy10.0
34waldenstrom macroglobulinemia10.0
35epithelioid hemangioendothelioma10.0FLT4, CD34
36pyogenic granuloma10.0CD34, FLT4
37thrombocytopenia10.0FLI1, CD34
38factor v leiden thrombophilia10.0CD34, FLI1
39endometrial carcinoma10.0CD34, FLT4
40non-hodgkin lymphoma10.0CD34, FLI1
41stomach cancer10.0CD34, FLT4
42vascular cancer9.9FLT4, CD34, FLI1
43angiosarcoma9.9CD34, FLT4, FLI1
44burkitt's lymphoma9.9CD34, FLI1, FLT4
45adenocarcinoma9.9FLT4, FLI1, CD34
46melanoma9.9CD34, FLT4, FLI1
47prostate cancer9.9FLT4, CD34, FLI1
48breast cancer9.9CD34, PROX1, FLT4, FLI1

Graphical network of the top 20 diseases related to Kaposiform Hemangioendothelioma:



Diseases related to kaposiform hemangioendothelioma

Symptoms for Kaposiform Hemangioendothelioma

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Drugs & Therapeutics for Kaposiform Hemangioendothelioma

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Kaposiform Hemangioendothelioma

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Anatomical Context for Kaposiform Hemangioendothelioma

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34MalaCards
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MalaCards organs/tissues related to Kaposiform Hemangioendothelioma:

34
Skin, Endothelial, Tonsil, Cervix, Bone, Breast, Kidney, Thymus, Spleen, Tongue

Animal Models for Kaposiform Hemangioendothelioma or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Kaposiform Hemangioendothelioma:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.8PROX1, FLT4, FLI1
2MP:00053708.5FLI1, FLT4, PROX1
3MP:00053877.9CD34, PROX1, FLT4, FLI1

Publications for Kaposiform Hemangioendothelioma

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53PubMed
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Articles related to Kaposiform Hemangioendothelioma:

(show top 50)    (show all 86)
idTitleAuthorsYear
1
Paclitaxel-based chemotherapy for aggressive kaposiform hemangioendothelioma of the temporomastoid region: Case report and review of the literature. (22907922)
2013
2
Microscopic Kaposiform hemangioendothelioma with extensive lymphangiomatosis: an extraordinary example of an unusual entity. (23129838)
2013
3
Congenital Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon Successfully Treated with Low-Dose Radiation Therapy. (23458157)
2013
4
Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: ultrastructural observation and immunohistochemistry staining reveal the trapping of blood components. (24134598)
2013
5
Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. (22871490)
2013
6
Kaposiform Hemangioendothelioma Infiltrates the Gut Wall: A Rare Case Report. (23389498)
2013
7
Kaposiform hemangioendothelioma in the uterine cervix of a 5-year girl. (22432747)
2012
8
Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon. (22648868)
2012
9
Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: from vincristine to sirolimus. (23070861)
2012
10
Capillary-lymphatic malformation, kaposiform hemangioendothelioma and delayed Kasabach-Merritt phenomenon. (20403115)
2011
11
Massive congenital kaposiform hemangioendothelioma of the eyelid in a neonate. (22134318)
2011
12
Kaposiform hemangioendothelioma of the breast in an adult female. (21570000)
2011
13
Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report. (21605441)
2011
14
Kaposiform hemangioendothelioma of the spleen in an adult: an initial case report. (21190143)
2011
15
Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment. (21516018)
2011
16
Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma. (20975337)
2010
17
Treatment of childhood kaposiform hemangioendothelioma with sirolimus. (20730884)
2010
18
Kaposiform hemangioendothelioma of the choledochus. (20850638)
2010
19
Kaposiform hemangioendothelioma of the kidney: an unusual presentation of a rare vascular neoplasm. (20588198)
2010
20
Vincristine and corticosteroids as first-line treatment of Kasabach-Merritt syndrome in kaposiform hemangioendothelioma. (19426625)
2009
21
Mediastinal and neck kaposiform hemangioendothelioma: report of three cases. (19706099)
2009
22
Adult-onset kaposiform hemangioendothelioma in a posttraumatic site. (19325356)
2009
23
Kaposiform hemangioendothelioma of the tongue in an adult. (19687407)
2009
24
Kaposiform hemangioendothelioma (with Kasabach Merritt syndrome) of the head and neck: case report and review of the literature. (19643504)
2009
25
Kaposiform hemangioendothelioma. (19327597)
2009
26
Kaposiform hemangioendothelioma: a rare cause of spontaneous hemothorax in infancy. Review of the literature. (18206444)
2008
27
Kaposiform hemangioendothelioma with distant lymphangiomatosis without an association to Kasabach-Merritt-Syndrome in a female adult! (18629354)
2008
28
Prox-1 promotes invasion of kaposiform hemangioendotheliomas. (18580962)
2008
29
Kaposiform hemangioendothelioma of the mediastinum in a 7-month-old boy: a case report. (16863862)
2006
30
Kaposiform hemangioendothelioma arising from the internal auditory canal. (16611794)
2006
31
Usefulness of D2-40 immunohistochemistry for differentiation between kaposiform hemangioendothelioma and tufted angioma. (16872472)
2006
32
Kaposiform hemangioendothelioma in an adult with an unusual presentation. (16684311)
2006
33
D2-40 immunohistochemical analysis of pediatric vascular tumors reveals positivity in kaposiform hemangioendothelioma. (15920541)
2005
34
Kaposiform hemangioendothelioma: the first reported case in a nonhuman animal species. (16006617)
2005
35
Pathology quiz case 2. Diagnosis: Kaposiform hemangioendothelioma (KHE). (15313873)
2004
36
Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops. (15163230)
2004
37
Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. (15105642)
2004
38
Transformation between Kaposiform hemangioendothelioma and tufted angioma. (12771476)
2003
39
Kaposiform hemangioendothelioma associated with Milroy's disease (primary hereditary lymphedema). (12861592)
2003
40
Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach-Merritt syndrome. (14507321)
2003
41
Successful nonoperative therapy for Kaposiform hemangioendothelioma involving the neck: report of 1 case. (14595290)
2003
42
Kaposiform hemangioendothelioma and therapy with interferon-alpha. (14595731)
2003
43
Kaposiform hemangioendothelioma of the external auditory canal in an adult. (12112561)
2002
44
A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging. (11727024)
2001
45
Kaposiform hemangioendothelioma: a locally aggressive vascular tumor. (9591789)
1998
46
Kasabach-Merritt syndrome-associated kaposiform hemangioendothelioma successfully treated with cyclophosphamide, vincristine, and actinomycin D. (9856681)
1998
47
Kaposiform hemangioendothelioma in adults. Clinicopathologic and immunohistochemical analysis of three cases. (9322599)
1997
48
Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. (9385948)
1997
49
Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: successful treatment with alpha-2A interferon. (9121401)
1997
50
Kaposiform hemangioendothelioma. An aggressive, locally invasive vascular tumor that can mimic hemangioma of infancy. (9420544)
1997

Variations for Kaposiform Hemangioendothelioma

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Expression for genes affiliated with Kaposiform Hemangioendothelioma

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Kaposiform Hemangioendothelioma

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Pathways for genes affiliated with Kaposiform Hemangioendothelioma

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51PathCards, 5Cell Signaling Technology
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Pathways related to Kaposiform Hemangioendothelioma according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.1CD34, FLI1

Compounds for genes affiliated with Kaposiform Hemangioendothelioma

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46Novoseek
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Compounds related to Kaposiform Hemangioendothelioma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1paraffin469.1CD34, FLT4
2vegf468.2CD34, PROX1, FLT4

GO Terms for genes affiliated with Kaposiform Hemangioendothelioma

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17Gene Ontology
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Biological processes related to Kaposiform Hemangioendothelioma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1lymphangiogenesisGO:0019469.3PROX1, FLT4
2positive regulation of cell proliferationGO:0082849.3PROX1, FLT4
3positive regulation of endothelial cell migrationGO:0105959.2PROX1, FLT4
4positive regulation of endothelial cell proliferationGO:0019389.0PROX1, FLT4

Products for genes affiliated with Kaposiform Hemangioendothelioma

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  • Antibodies
  • Proteins
  • Lysates
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Sources for Kaposiform Hemangioendothelioma

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet