KH
MCID: KPS002
MIFTS: 34

Kaposiform Hemangioendothelioma (KH) malady

Cardiovascular, Fetal, Cancer categories

Summaries for Kaposiform Hemangioendothelioma

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33MalaCards
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MalaCards: Kaposiform Hemangioendothelioma, also known as congenital cutaneous multifocal kaposiform hemangioendothelioma, is related to hemangioendothelioma and kaposi's sarcoma. An important gene associated with Kaposiform Hemangioendothelioma is FLT4 (fms-related tyrosine kinase 4), and among its related pathways is Lymphocyte Signaling. The compounds estrogen and vegf have been mentioned in the context of this disorder. Affiliated tissues include spleen, thymus and kidney, and related mouse phenotype immune system.

Aliases & Classifications for Kaposiform Hemangioendothelioma

Sources:
43NIH Rare Diseases, 49Orphanet, 61UMLS, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 36MESH via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Fetal, Cancer
Anatomical: Cardiovascular


Characteristics (Orphanet epidemiological data):

49
kaposiform hemangioendothelioma:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

kaposiform hemangioendothelioma 43 49 61
congenital cutaneous multifocal kaposiform hemangioendothelioma 43
kaposiform hemangio-endothelioma 43
khe 43
kh 43


External Ids:

ICD10 via Orphanet26 D18.0
SNOMED-CT via Orphanet58 403983000
UMLS via Orphanet62 C1367420
MESH via Orphanet36 C537007

Related Diseases for Kaposiform Hemangioendothelioma

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17GeneCards, 18GeneDecks
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Diseases related to Kaposiform Hemangioendothelioma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 54)
idRelated DiseaseScoreTop Affiliating Genes
1hemangioendothelioma31.7IFNA2, CD34, FLI1, FLT4
2kaposi's sarcoma30.3CD34
3acute leukemia29.9FLT4, CD34
4tufted angioma10.6
5lymphangiomatosis10.5
6n syndrome10.4
7hereditary lymphedema10.2
8adult syndrome10.2
9hemophilia10.2
10hemangioma thrombocytopenia syndrome10.2
11soft tissue sarcoma10.2
12lymphatic malformation10.2
13lymphangiosarcoma10.0PROX1
14malignant mesothelioma10.0FLT4
15pancreatic cancer10.0FLT4
16capillary hemangioma10.0CD34
17myeloid leukemia10.0CD34
18hemangiopericytoma10.0CD34
19myelodysplastic syndromes10.0CD34
20myelofibrosis10.0IFNA2
21mycosis fungoides10.0IFNA2
22papilloma10.0FLT4, IFNA2
23polycythemia10.0CD34
24lymphedema10.0FLT4, PROX1
25pyogenic granuloma10.0FLT4, CD34
26hepatocellular carcinoma10.0CD34, IFNA2
27lymphangioma10.0FLT4, PROX1, CD34
28epithelioid hemangioendothelioma10.0CD34, FLT4, IFNA2
29liposarcoma10.0FLI1, CD34
30colorectal cancer10.0FLT4, IFNA2, CD34
31angiosarcoma10.0FLT4, FLI1, CD34
32sarcoma10.0FLI1, FLT4, CD34
33thrombocytopenia10.0CD34, FLI1, IFNA2
34macroglobulinemia10.0
35presbyopia10.0
36muscular atrophy10.0
37amyloidosis10.0
38blood protein disease10.0
39brain stem infarction10.0
40spinal muscular atrophy10.0
41survival motor neuron spinal muscular atrophy10.0
42waldenstrom macroglobulinemia10.0
43nova syndrome10.0
44brain injury10.0
45inflammatory bowel disease 1010.0
46inflammatory bowel disease 110.0
47synovial sarcoma10.0CD34, FLI1
48burkitt's lymphoma10.0FLT4, FLI1, CD34
49adenocarcinoma10.0CD34, FLI1, FLT4
50prostate cancer10.0FLI1, FLT4, CD34

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



Diseases related to kaposiform hemangioendothelioma

Clinical Features for Kaposiform Hemangioendothelioma

Drugs & Therapeutics for Kaposiform Hemangioendothelioma

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Kaposiform Hemangioendothelioma

Drug clinical trials:

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Search CenterWatch for Kaposiform Hemangioendothelioma

Genetic Tests for Kaposiform Hemangioendothelioma

Anatomical Context for Kaposiform Hemangioendothelioma

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33MalaCards
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MalaCards organs/tissues related to Kaposiform Hemangioendothelioma:

33
Spleen, Thymus, Kidney, Breast, Skin, Cervix, Tonsil, Endothelial, Tongue

Animal Models for Kaposiform Hemangioendothelioma or affiliated genes

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

MGI Mouse Phenotypes related to Kaposiform Hemangioendothelioma:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053878.1CD34, PROX1, FLT4, FLI1

Publications for Kaposiform Hemangioendothelioma

Sources:
51PubMed
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Articles related to Kaposiform Hemangioendothelioma:

(show top 50)    (show all 88)
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
Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: ultrastructural observation and immunohistochemistry staining reveal the trapping of blood components. (24134598)
2013
4
Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. (22871490)
2013
5
Successful Treatment of Kasabach-Merritt Phenomenon Arising From Kaposiform Hemangioendothelioma by Sirolimus. (24309604)
2013
6
Rapidly enlarging "bruise" on the back of an infant. Kaposiform hemangioendothelioma complicated by Kasabach-Merritt syndrome. (24005816)
2013
7
Mediastinal Kaposiform Hemangioendothelioma and Kasabach-Merritt Phenomenon in a Patient with no Skin Changes and a Normal Chest CT. (24047193)
2013
8
Kaposiform hemangioendothelioma in the uterine cervix of a 5-year girl. (22432747)
2012
9
Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: successful treatment with vincristine and ticlopidine. (22161581)
2012
10
Successful treatment of Kasabach-Merritt syndrome arising from kaposiform hemangioendothelioma by systemic corticosteroid therapy and surgery. (21947597)
2012
11
Intra-abdominal Kaposiform hemangioendothelioma and the benefits of laparoscopic surveillance. (22431247)
2012
12
Massive congenital kaposiform hemangioendothelioma of the eyelid in a neonate. (22134318)
2011
13
Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report. (21605441)
2011
14
Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment. (21516018)
2011
15
Kaposiform hemangioendothelioma with adolescent thoracic scoliosis: a case report and review of literature. (21387193)
2011
16
Primary kaposiform hemangioendothelioma of a long bone: two cases in unusual locations with long-term follow up. (21615616)
2011
17
Treatment of childhood kaposiform hemangioendothelioma with sirolimus. (20730884)
2010
18
Successful treatment of kaposiform hemangioendothelioma and tufted angioma with vincristine. (20523249)
2010
19
Vincristine and corticosteroids as first-line treatment of Kasabach-Merritt syndrome in kaposiform hemangioendothelioma. (19426625)
2009
20
Mediastinal and neck kaposiform hemangioendothelioma: report of three cases. (19706099)
2009
21
Kaposiform hemangioendothelioma (with Kasabach Merritt syndrome) of the head and neck: case report and review of the literature. (19643504)
2009
22
Kaposiform hemangioendothelioma. (19327597)
2009
23
Successful management of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine. (19706115)
2009
24
Refractory kaposiform hemangioendothelioma that expressed vascular endothelial growth factor receptor (VEGFR)-2 and VEGFR-3: a case report. (19262246)
2009
25
Mediastinal Kaposiform hemangioendothelioma and Kasabach-Merritt phenomenon in an infant: treatment with interferon. (19687760)
2009
26
Intracranial kaposiform hemangioendothelioma: proposal of a new malignant variant. (19278316)
2009
27
Kaposiform hemangioendothelioma: a rare cause of spontaneous hemothorax in infancy. Review of the literature. (18206444)
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
Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon. (15793511)
2005
36
A case of axillary kaposiform hemangioendothelioma resembles a soft tissue sarcoma. (16282890)
2005
37
Pathology quiz case 2. Diagnosis: Kaposiform hemangioendothelioma (KHE). (15313873)
2004
38
Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. (15105642)
2004
39
Transformation between Kaposiform hemangioendothelioma and tufted angioma. (12771476)
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
A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging. (11727024)
2001
44
Additional case of kaposiform hemangioendothelioma in an adult. (11669025)
2001
45
Kaposiform hemangioendothelioma in adult. Report of a case with amianthoid-like fibrosis and angiectases. (11378910)
2000
46
Congenital cutaneous multifocal kaposiform hemangioendothelioma. (10608250)
1999
47
Kasabach-Merritt syndrome-associated kaposiform hemangioendothelioma successfully treated with cyclophosphamide, vincristine, and actinomycin D. (9856681)
1998
48
Kaposiform hemangioendothelioma in adults. Clinicopathologic and immunohistochemical analysis of three cases. (9322599)
1997
49
Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. (9385948)
1997
50
Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: successful treatment with alpha-2A interferon. (9121401)
1997

Genetic Variations for Kaposiform Hemangioendothelioma

Expression for genes affiliated with Kaposiform Hemangioendothelioma

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Kaposiform Hemangioendothelioma

Search GEO for disease gene expression data for Kaposiform Hemangioendothelioma.

Pathways for genes affiliated with Kaposiform Hemangioendothelioma

Sources:
4Cell 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

Sources:
45Novoseek
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Compounds related to Kaposiform Hemangioendothelioma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1estrogen458.7CD34, FLT4, IFNA2
2vegf457.8CD34, PROX1, FLT4, IFNA2

GO Terms for genes affiliated with Kaposiform Hemangioendothelioma

Sources:
16Gene Ontology
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Biological processes related to Kaposiform Hemangioendothelioma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1lymphangiogenesisGO:0019469.4PROX1, FLT4
2positive regulation of endothelial cell migrationGO:0105959.3PROX1, FLT4
3negative regulation of gene expressionGO:0106299.1CD34, IFNA2
4positive regulation of endothelial cell proliferationGO:0019389.1PROX1, FLT4

Products for genes affiliated with Kaposiform Hemangioendothelioma

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Sources for Kaposiform Hemangioendothelioma

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