Kaposi sarcoma, susceptibility to

Preferred Label : Kaposi sarcoma, susceptibility to;

CISMeF acronym : MCD;

Type : Phenotype, molecular basis known;

Alternative titles and symbols : Multiple idiopathic pigmented hemangiosarcoma, susceptibility to;

Included titles and symbols : Multicentric castleman disease, susceptibility to; MCD;

Description : Kaposi sarcoma (KS) is an invasive angioproliferative inflammatory condition that occurs commonly in men infected with human immunodeficiency virus (HIV; see 609423). In the early stages of KS, lesions appear reactive and are stimulated to grow by the actions of inflammatory cytokines and growth factors. In the late stages of KS, a malignant phenotype that appears to be monoclonal can develop. Infection with human herpesvirus-8 (HHV-8), also known as KS-associated herpesvirus (KSHV), is necessary but not sufficient for KS development. Coinfection with HIV markedly increases the likelihood of KS development, and additional environmental, hormonal, and genetic cofactors likely contribute to its pathogenesis (summary by Foster et al., 2000). Suthaus et al. (2012) noted that HHV-8 is the etiologic agent not only of KS, but also of primary effusion lymphoma and plasma cell-type multicentric Castleman disease (MCD).;

Inheritance : Autosomal dominant;

Prefixed ID : #148000;

Details

Origin ID

148000;

UMLS CUI

C3538945;

Automatic exact mappings (from CISMeF team)
- Kaposi Sarcoma [NCIt concept]
- Kaposi sarcoma [PASCAL descriptor]
- Kaposi sarcoma [ORDO Disease]
- Kaposi sarcoma [Radlex concept]
- Kaposi's sarcoma [ICD-10 category]
- Kaposi's sarcoma [HRDO Sign]
- Kaposi's sarcoma [ICD-9 code]
- Kaposi's sarcoma [MedDRA Preferred Term]
- Kaposi's sarcoma [HPO term]
- Kaposi's sarcoma (disorder) [SNOMED CT concept]
- Kaposi's sarcoma NOS [MedDRA LLT]
- Kaposi's sarcoma, morphology (morphologic abnormality) [SNOMED CT concept]
- Kaposi's sarcomas [MedDRA High Level Term]
- Kaposis sarcoma [ICD-10 category (who)]
- MUC1 wt Allele [NCIt concept]
- Macular corneal dystrophy [ORDO Disease]
- Multicentric Castleman disease [ORDO Disease]
- Multiple carboxylase deficiency [ORDO Disease]
- kaposi sarcoma [MedlinePlus Topic]
- kaposi's sarcoma [SNOMED Notion]
- macular dystrophy, corneal type 1 [MeSH Supplementary Concept]
- malonyl-CoA decarboxylase [ORDO Gene]
- mucin 1, cell surface associated [ORDO Gene]
- multicentric Castleman disease [DO Concept]
- sarcoma, kaposi [MeSH Descriptor]
- sarcoma, kaposi [MeSH concept]
Genes related to phenotype
- Interleukin 6 [OMIM gene]
HPO term(s)
- Autosomal dominant inheritance [HPO term]
- Edema [HPO term]
- Hypermelanotic macule [HPO term]
- Kaposi's sarcoma [HPO term]
- Neoplasm [HPO term]
- macules [HPO term]
ORDO concept(s)
- Castleman disease [ORDO Disease]
- Kaposi sarcoma [ORDO Disease]
Semantic type(s)
- Finding [UMLS semantic type]

Keyword's position in hierarchy(ies) :

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