Abstract
Kaposi sarcoma (KS) is the most common HIV-associated malignancy in children and adolescents in Africa. Pediatric KS is distinct from adult disease. We evaluated the clinical characteristics associated with long-term outcomes. We performed a retrospective observational analysis of 70 HIV-infected children and adolescents with KS less than 18 years of age diagnosed between 8/2010 and 6/2013 in Lilongwe, Malawi. Local first-line treatment included bleomycin and vincristine plus nevirapine-based highly active anti-retroviral therapy (HAART). Median age was 8.6 years (range 1.7–17.9); there were 35 females (50%). Most common sites of presentation were: lymph node (74%), skin (59%), subcutaneous nodules (33%), oral (27%), woody edema (24%), and visceral (16%). Eighteen (26%) presented with lymphadenopathy only. Severe CD4 suppression occurred in 28%. At time of KS diagnosis, 49% were already on HAART. Overall, 28% presented with a platelet count < 100 x 109/L and 37% with hemoglobin < 8 g/dL. The 2-year event-free (EFS) and overall survival (OS) were 46% and 58% respectively (median follow-up 29 months, range 15–50). Multivariable analysis of risk of death and failure to achieve EFS demonstrated that visceral disease (odds ratios [OR] 19.08 and 11.61, 95% CI 2.22–163.90 and 1.60–83.95 respectively) and presenting with more than 20 skin/oral lesions (OR 9.57 and 22.90, 95% CI 1.01–90.99 and 1.00–524.13 respectively) were independent risk factors for both. Woody edema was associated with failure to achieve EFS (OR 7.80, 95% CI 1.84–33.08) but not death. Univariable analysis revealed that lymph node involvement was favorable for EFS (OR 0.28, 95% CI 0.08–0.99), while T1 TIS staging criteria, presence of cytopenias, and severe immune suppression were not associated with increased mortality. Long-term complete remission is achievable in pediatric KS, however outcomes vary according to clinical presentation. Based on clinical heterogeneity, treatment according to risk-stratification is necessary to improve overall outcomes.
Highlights
Kaposi sarcoma (KS) is the most common human immunodeficiency virus (HIV)-associated malignancy in children and adolescents in sub-Saharan Africa.[1]
We evaluated the clinical characteristics of HIV-infected children and adolescents with KS in Lilongwe, Malawi and identified specific clinical factors that influence survival outcomes in patients receiving bleomycin and vincristine (BV) in combination with highly active antiretroviral therapy (HAART)
70 HIV-infected children and adolescents < 18 years of age were diagnosed with KS at the Malawi Clinical Centre of Excellence (MW-COE) and Kamuzu Central Hospital (KCH). (Table 1) Using a denominator of children initiated on HAART at the MW-COE as a proxy for number of new pediatric HIV diagnoses during that time period, KS represented 5.2% (70/1,359) of children and adolescents newly diagnosed with HIV infection
Summary
Kaposi sarcoma (KS) is the most common human immunodeficiency virus (HIV)-associated malignancy in children and adolescents in sub-Saharan Africa.[1]. KS is caused by human herpesvirus-8 (HHV-8) infection, a virus with prevalence rates that vary geographically.[2]. Eastern and central Africa in particular have the highest prevalence of HHV-8 infection in the world.[3–9]. KS has become an important and widespread complication of the HIV epidemic in sub-Saharan Africa, affecting adults, but children and adolescents as well. While clinical descriptions and treatment paradigms for adult KS have been well established, there exist only a few clinical descriptions of pediatric KS cohorts in Africa.[10–14]. Since the largest published series on HIV-associated malignancies from the United States or Europe reported only eight pediatric KS patients from 1978–1996 in the AIDS-Cancer Match Registry Study Group, the pre-highly active antiretroviral therapy (HAART) era in the western world fails to serve as a guide.[15–20]
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