Abstract
BackgroundEndemic Kaposi sarcoma (KS) was first described in African children over fifty years ago, but has recently been overshadowed by HIV-related disease. We aimed to evaluate the similarities and differences between endemic HIV-negative and epidemic HIV-positive pediatric KS in a KS-associated herpesvirus-endemic region of Africa.MethodsWe describe clinical characteristics of 20 HIV-negative children with endemic KS over a six-year period and compare findings with a historical control—an HIV-related pediatric KS cohort from Lilongwe, Malawi.ResultsThe HIV-negative endemic KS cohort was 70% male with a median age of 9.3 years. Lymph node involvement was present in 50%, hyperpigmented skin lesions in 45%, and woody edema in 40%. One patient (5%) presented with oral KS involvement and no patients presented initially with visceral KS. Significant anemia (hemoglobin < 8 g/dL) and thrombocytopenia (platelet count < 100 × 109/L) were found at time of original KS diagnosis in 45 and 40% respectively. In both HIV-negative and HIV-positive cohorts, lymphadenopathy was the most common presentation, prototypical skin lesions were often absent, severe cytopenias were a common clinical feature, and treatment outcomes were similar. Patients with endemic KS demonstrated less frequent oral involvement (5% versus 29%, P = 0.03) and a lower proportion of patients with visceral involvement (0% versus 16%, P = 0.06).ConclusionsThese data suggest clinical overlap between epidemiological variants. Treatment protocols for pediatric KS in sub-Saharan Africa should be devised to include both endemic HIV-negative and epidemic HIV-related disease to better define the clinical and biological comparison.
Highlights
Descriptions of endemic Kaposi sarcoma (KS) of childhood originated more than fifty years ago in central and eastern Africa—decades before the emergence of HIV—offering details of the clinical characteristics and outcomes in HIV-negative African children living in regions with endemic human herpesvirus-8/KS-associated herpesvirus (KSHV) infection [1,2,3,4,5]
Patients were staged according to the Lilongwe Pediatric KS Staging Classification, a pediatric-specific staging paradigm that attempts to address the unique clinical features of childhood KS observed in KSHV-endemic regions of Africa [17, 19]
There were 140 new HIV-related pediatric KS diagnoses over the same six-year time period [20]
Summary
Descriptions of endemic Kaposi sarcoma (KS) of childhood originated more than fifty years ago in central and eastern Africa—decades before the emergence of HIV—offering details of the clinical characteristics and outcomes in HIV-negative African children living in regions with endemic human herpesvirus-8/KS-associated herpesvirus (KSHV) infection [1,2,3,4,5]. Unique features of endemic pediatric KS that were distinct from adults included frequent presentation primarily with lymph node disease, less frequent skin involvement, and a fulminant clinical course if untreated—especially for children with lymphadenopathic KS [3, 4]. Following those initial descriptions, little has been published on endemic HIV-unrelated KS [6,7,8,9].
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