Prognosis

Classical KS typically runs an indolent course over years or decades, with gradual development of new lesions and complications such as lower-limb lymphoedema. An Increased risk of developing a second malignancy, usually non-Hodgkin's lymphoma, has been reported. Endemic (African) KS may run an indolent course similar to classical KS, with nodules and plaques in association with lower limb oedema. The lymphadenopathic form of African KS in children has an aggressive course and carries a poor prognosis. Epidemic AIDS-related KS may be a disseminated and fulminant disease. The prognosis is determined by the extent of tumour, severity of immunodeficiency and the presence or absence of systemic illness. Each of these variables is independently associated with survival and has resulted in the prospectively validated tumour, immunodeficiency and systemic illness (TIS) staging classification (see Table 29.1).9 Immune status is the most important prognostic factor, and patients with a CD4 count greater than 200 x 106 cells/litre have a better prognosis. Opportunistic infections are often the cause of death in this group of patients. However, with the advent of HAART and better prophylaxis of opportunistic infections, the prognosis of AIDS-associated KS may be improving although newer therapies specifically for KS have not been shown to improve overall survival.

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