Fisiopatologia

5 Subtypes

Associated with pulmonary Kaposi Sarcoma which may be fatal due to immune reconstitution inflammatory syndrome - ask about pulmonary symptoms and check X ray
Pedir gastro - se tiver lesões orais fazer endoscopia e colonoscopia
Epidemic HIV-associated KS is the most common AIDS-defining neoplasm and most common KS subtype. Disease course varies from stable single lesions to rapidly fatal progression. It primarily affects severely immunodeficient HIV patients without adequate antiretroviral therapy (ART). KS typically regresses after starting ART, though IRIS may cause severe presentations or new-onset cases.
Despite effective ART, KS risk remains 30-80 times higher in people living with HIV compared to the general population. Immunosenescence from HIV-related aging may contribute to KS development. Mortality correlates inversely with CD4 count and consistent ART use.
Risk factors: low CD4 count
Clinical presentation

Predominantly in men (male:female ratio 2:1 to 17:1) of Eastern European and Mediterranean origin.
After age 60, advanced age
prior HHV-8 infection

HHV is 1-5% positive in western population vs 40-80% in certain regions of Africa
equatorial african countries, more aggressive
Endemic KS (not HIV-associated) occurs in equatorial Africa, particularly among children and adolescent males. Four clinical courses exist:

Benign: Slow-progressing skin nodules similar to classic KS, mainly in middle-aged men
Locally aggressive: Cutaneous lesions infiltrating soft tissue and bone; fatal within 5-7 years
Diffusely aggressive: Widespread mucocutaneous and visceral involvement with poor prognosis
Fulminant: Primarily lymphadenopathy and visceral involvement; aggressive progression; mainly in young children

Endemic KS represented 9% of Central African cancers before the HIV pandemic dramatically increased KS incidence in sub-Saharan Africa.

Transplant-associated - similar to AIDS, can regress with decreased immunosuppression
Iatrogenic KS can occur during long-term immunosuppression and may improve with reduced immunosuppressive therapy. It affects over 5% of organ transplant patients who develop malignancies, with a 50-500 times higher risk compared to the general population.

KS has been increasingly reported in younger HIV-negative MSM from regions with low HHV-8 seroprevalence (France, England, Germany). Like classic KS, it follows an indolent course with widespread skin lesions but rare visceral involvement. CD4 count and CD4/CD8 ratio inversely correlate with disease severity. Due to these distinctive features, it's now classified as a fifth epidemiologic subtype.

Sarcoma de Kaposi