KH
MCID: KPS002
MIFTS: 40

Kaposiform Hemangioendothelioma (KH) malady

Rare diseases, Cardiovascular diseases, Fetal diseases, Cancer diseases categories
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Summaries for Kaposiform Hemangioendothelioma

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MalaCards based summary: Kaposiform Hemangioendothelioma, also known as congenital cutaneous multifocal kaposiform hemangioendothelioma, is related to hemangioendothelioma and lymphedema. 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 bone, and related mouse phenotypes are cardiovascular system and liver/biliary system.

Aliases & Classifications for Kaposiform Hemangioendothelioma

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Sources:
42NIH Rare Diseases, 48Orphanet, 62UMLS, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Kaposiform Hemangioendothelioma, Aliases & Descriptions:

Name: Kaposiform Hemangioendothelioma 42 48 62
Congenital Cutaneous Multifocal Kaposiform Hemangioendothelioma 42 62
Kaposiform Hemangio-Endothelioma 42 62
 
Khe 42
Kh 42


Classifications:



Characteristics (Orphanet epidemiological data):

48
kaposiform hemangioendothelioma:
Inheritance: Sporadic; Age of onset: Variable


External Ids:

MESH via Orphanet35 C537007
ICD10 via Orphanet26 D18.0
UMLS via Orphanet63 C1367420

Related Diseases for Kaposiform Hemangioendothelioma

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

(show all 49)
idRelated DiseaseScoreTop Affiliating Genes
1hemangioendothelioma31.5FLI1, FLT4, CD34
2lymphedema30.5PROX1, FLT4
3hemangioma30.1FLI1, CD34, PROX1, FLT4
4lymphangioma30.0PROX1, FLT4, CD34
5sarcoma30.0FLI1, FLT4, CD34
6acute leukemia29.6FLT4, CD34
7leukemia29.5FLT4, FLI1, CD34
8tufted angioma10.7
9mediastinitis10.5
10lymphangiomatosis10.5
11endotheliitis10.4
12lymphangiosarcoma10.4PROX1
13fragile x syndrome10.3
14hereditary lymphedema10.2
15intussusception10.2
16tonsillitis10.2
17cervicitis10.2
18laryngitis10.2
19pericardial effusion10.2
20hemophilia10.2
21hemangioma thrombocytopenia syndrome10.2
22laryngomalacia10.2
23soft tissue sarcoma10.2
24kaposi's sarcoma10.2CD34
25spinal muscular atrophy10.0
26waldenstrom macroglobulinemia10.0
27muscular atrophy10.0
28presbyopia10.0
29macroglobulinemia10.0
30neuronitis10.0
31amyloidosis10.0
32brain stem infarction10.0
33inflammatory bowel disease10.0
34synovial sarcoma10.0FLI1, CD34
35liposarcoma10.0FLI1, CD34
36epithelioid hemangioendothelioma10.0FLT4, CD34
37pyogenic granuloma10.0CD34, FLT4
38thrombocytopenia10.0FLI1, CD34
39factor v leiden thrombophilia9.9CD34, FLI1
40endometrial carcinoma9.8CD34, FLT4
41non-hodgkin lymphoma9.8CD34, FLI1
42stomach cancer9.8CD34, FLT4
43vascular cancer9.8FLT4, CD34, FLI1
44angiosarcoma9.8CD34, FLT4, FLI1
45burkitt's lymphoma9.7CD34, FLI1, FLT4
46adenocarcinoma9.7FLT4, FLI1, CD34
47melanoma9.7CD34, FLT4, FLI1
48prostate cancer9.7FLT4, CD34, FLI1
49breast cancer9.5CD34, 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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Drug clinical trials:

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Search NIH Clinical Center for Kaposiform Hemangioendothelioma

Genetic Tests for Kaposiform Hemangioendothelioma

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Anatomical Context for Kaposiform Hemangioendothelioma

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MalaCards organs/tissues related to Kaposiform Hemangioendothelioma:

32
Skin, Endothelial, Bone, Spleen, Thymus, Kidney, Breast, Cervix, Tonsil, Tongue

Animal Models for Kaposiform Hemangioendothelioma or affiliated genes

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MGI Mouse Phenotypes related to Kaposiform Hemangioendothelioma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.8FLI1, FLT4, PROX1
2MP:00053708.5FLT4, PROX1, FLI1
3MP:00053877.9FLT4, CD34, PROX1, FLI1

Publications for Kaposiform Hemangioendothelioma

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Articles related to Kaposiform Hemangioendothelioma:

(show top 50)    (show all 99)
idTitleAuthorsYear
1
CyberKnife Treatment for Kaposiform Hemangioendothelioma of the Ilium in an adult: case report and review of the literature. (25138302)
2014
2
Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome mistaken for child abuse in a newborn. (24738105)
2014
3
Infantile hemangioma or kaposiform hemangioendothelioma? (24745913)
2014
4
A rare case of kaposiform hemangioendothelioma presenting as intussusception in a 4-month-old child without Kasabach-Merrit syndrome: A case report. (25336808)
2014
5
Successful treatment of kaposiform hemangioendothelioma with everolimus. (25306933)
2014
6
Multifocal kaposiform hemangioendothelioma in multiple visceral organs: an autopsy of 9-day-old female baby. (24931465)
2014
7
Paclitaxel-based chemotherapy for aggressive kaposiform hemangioendothelioma of the temporomastoid region: Case report and review of the literature. (22907922)
2013
8
Microscopic Kaposiform hemangioendothelioma with extensive lymphangiomatosis: an extraordinary example of an unusual entity. (23129838)
2013
9
Congenital Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon Successfully Treated with Low-Dose Radiation Therapy. (23458157)
2013
10
Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: ultrastructural observation and immunohistochemistry staining reveal the trapping of blood components. (24134598)
2013
11
Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. (22871490)
2013
12
Kaposiform hemangioendothelioma in the uterine cervix of a 5-year girl. (22432747)
2012
13
Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon. (22648868)
2012
14
Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: from vincristine to sirolimus. (23070861)
2012
15
Capillary-lymphatic malformation, kaposiform hemangioendothelioma and delayed Kasabach-Merritt phenomenon. (20403115)
2011
16
Massive congenital kaposiform hemangioendothelioma of the eyelid in a neonate. (22134318)
2011
17
Kaposiform hemangioendothelioma of the breast in an adult female. (21570000)
2011
18
Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report. (21605441)
2011
19
Kaposiform hemangioendothelioma of the spleen in an adult: an initial case report. (21190143)
2011
20
Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment. (21516018)
2011
21
Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma. (20975337)
2010
22
Treatment of childhood kaposiform hemangioendothelioma with sirolimus. (20730884)
2010
23
Kaposiform hemangioendothelioma of the choledochus. (20850638)
2010
24
Kaposiform hemangioendothelioma of the kidney: an unusual presentation of a rare vascular neoplasm. (20588198)
2010
25
Vincristine and corticosteroids as first-line treatment of Kasabach-Merritt syndrome in kaposiform hemangioendothelioma. (19426625)
2009
26
Mediastinal and neck kaposiform hemangioendothelioma: report of three cases. (19706099)
2009
27
Adult-onset kaposiform hemangioendothelioma in a posttraumatic site. (19325356)
2009
28
Kaposiform hemangioendothelioma of the tongue in an adult. (19687407)
2009
29
Kaposiform hemangioendothelioma (with Kasabach Merritt syndrome) of the head and neck: case report and review of the literature. (19643504)
2009
30
Kaposiform hemangioendothelioma: a rare cause of spontaneous hemothorax in infancy. Review of the literature. (18206444)
2008
31
Kaposiform hemangioendothelioma with distant lymphangiomatosis without an association to Kasabach-Merritt-Syndrome in a female adult! (18629354)
2008
32
Prox-1 promotes invasion of kaposiform hemangioendotheliomas. (18580962)
2008
33
Kaposiform hemangioendothelioma of the mediastinum in a 7-month-old boy: a case report. (16863862)
2006
34
Kaposiform hemangioendothelioma arising from the internal auditory canal. (16611794)
2006
35
Usefulness of D2-40 immunohistochemistry for differentiation between kaposiform hemangioendothelioma and tufted angioma. (16872472)
2006
36
Kaposiform hemangioendothelioma in an adult with an unusual presentation. (16684311)
2006
37
D2-40 immunohistochemical analysis of pediatric vascular tumors reveals positivity in kaposiform hemangioendothelioma. (15920541)
2005
38
Pathology quiz case 2. Diagnosis: Kaposiform hemangioendothelioma (KHE). (15313873)
2004
39
Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops. (15163230)
2004
40
Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. (15105642)
2004
41
Kaposiform hemangioendothelioma associated with Milroy's disease (primary hereditary lymphedema). (12861592)
2003
42
Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach-Merritt syndrome. (14507321)
2003
43
Successful nonoperative therapy for Kaposiform hemangioendothelioma involving the neck: report of 1 case. (14595290)
2003
44
Kaposiform hemangioendothelioma of the external auditory canal in an adult. (12112561)
2002
45
A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging. (11727024)
2001
46
Kaposiform hemangioendothelioma: a locally aggressive vascular tumor. (9591789)
1998
47
Successful multimodal therapy for kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: case report and review of the literature. (9658430)
1998
48
Kasabach-Merritt syndrome-associated kaposiform hemangioendothelioma successfully treated with cyclophosphamide, vincristine, and actinomycin D. (9856681)
1998
49
Kaposiform hemangioendothelioma in adults. Clinicopathologic and immunohistochemical analysis of three cases. (9322599)
1997
50
Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. (9385948)
1997

Variations for Kaposiform Hemangioendothelioma

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Expression for genes affiliated with Kaposiform Hemangioendothelioma

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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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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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Sources:
44Novoseek
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Compounds related to Kaposiform Hemangioendothelioma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1paraffin449.1CD34, FLT4
2vegf448.2CD34, PROX1, FLT4

GO Terms for genes affiliated with Kaposiform Hemangioendothelioma

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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
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Sources for Kaposiform Hemangioendothelioma

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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet