AIDS-associated Kaposi sarcoma
See also in: External and Internal Eye,Anogenital,Oral Mucosal LesionSynopsis
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 human immunodeficiency virus (HIV)-seropositive patients and is an AIDS-defining illness. This form of KS is primarily seen in the MSM population, but it can be seen in female partners of men with the disease in addition to HIV-infected women. Lesions may worsen during immune reconstitution inflammatory syndrome and may also appear in patients with HIV who have received long-term antiretroviral therapy (ART).
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 (GI) 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 cells 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 tumor formation is noted, thousands of virion particles are assembled, resulting in cell lysis. HHV-8 requires additional cofactors for the development of KS. HIV coinfection acts as a stimulant for HHV-8 viral lytic expression and via its suppression of the immune system.
The introduction of ART dramatically decreased the incidence, morbidity, and mortality of AIDS-associated KS.
For discussion of classic, endemic, and iatrogenic forms of KS, see non-AIDS Kaposi sarcoma.
Codes
C46.9 – Kaposi's sarcoma, unspecified
SNOMEDCT:
420524008 – Kaposi's sarcoma associated with AIDS
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Last Updated:01/02/2024