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;
Origin ID : 148000;
UMLS CUI : C3538945;
Automatic exact mappings (from CISMeF team)
Genes related to phenotype
HPO term(s)
ORDO concept(s)
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