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AIDS-associated Kaposi sarcoma
See also in: External and Internal Eye,Anogenital,Oral Mucosal Lesion
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AIDS-associated Kaposi sarcoma

See also in: External and Internal Eye,Anogenital,Oral Mucosal Lesion
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Contributors: Levashni Naidoo MBChB, MMed, FCDerm, Ncoza C. Dlova MBChB, FCDerm, PhD, Anisa Mosam MBChB, MMed, FCDerm, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Kaposi sarcoma (KS) is a malignancy of vascular endothelial cells that occurs in several forms: classic KS, endemic KS, iatrogenically-induced KS, and human immunodeficiency virus (HIV)-associated KS. All 4 types can be linked to co-infection with human herpesvirus type 8 (HHV-8), and the cutaneous lesions are morphologically and histologically indistinguishable among the types. KS may be a reactive process rather than a true neoplasm because it does not produce conventional metastases but spreads in a multifocal way.

The outbreak of KS among young, previously healthy men who have sex with men (MSM) heralded the recognition of AIDS in 1981. AIDS-associated KS is the most common neoplasm in HIV-seropositive patients. It is an AIDS-defining illness. This form of KS disproportionately affects MSM, African Americans (regardless of sexual orientation), and heterosexual African individuals. KS lesions have been reported in up to 35% of AIDS patients and are seen more commonly in those with CD4 counts less than 150-200 cells/mm3.

Patients with AIDS-associated KS often have multifocal cutaneous disease. Around 20% of patients will have concomitant visceral involvement, which places these patients at risk for hemorrhage from gastrointestinal lesions, cardiac tamponade, and pulmonary obstruction. Additionally, AIDS-associated KS is more likely than classic KS to display a rapidly progressive course.

Spindle cells of endothelial origin are the predominant cell affected. In the latent phase, HHV-8 antigens promote cell proliferation by inactivating the RB gene, which leads to transcription of S-phase genes and blocks apoptosis via p53 and p27Kip1 suppression. In the lytic phase, when tumour formation is noted, thousands of virion particles are assembled resulting in cell lysis. HHV-8 requires additional co-factors for the development of KS. HIV co-infection acts as a stimulant for HHV-8 viral lytic expression and via its suppression of the immune system.

The introduction of highly active antiretroviral therapy (HAART) dramatically decreased the incidence, morbidity, and mortality of AIDS-associated KS.

For discussion of classic, endemic, and iatrogenic forms, see Non-AIDS Kaposi sarcoma.

Codes

ICD10CM:
C46.0 – Kaposi's sarcoma of skin

SNOMEDCT:
420524008 – Kaposi's sarcoma associated with AIDS

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Last Reviewed: 08/22/2017
Last Updated: 10/27/2017
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AIDS-associated Kaposi sarcoma
See also in: External and Internal Eye,Anogenital,Oral Mucosal Lesion
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AIDS-associated Kaposi sarcoma : Conjunctiva, Eyelids, Purple color, Firm nodules
Clinical image of AIDS-associated Kaposi sarcoma
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