Abstract
Background: Endemic 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. Methods: We 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. Results: The 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). Conclusions: These 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.
Original language | English (US) |
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Article number | 33 |
Journal | Infectious Agents and Cancer |
Volume | 13 |
Issue number | 1 |
DOIs | |
State | Published - Nov 9 2018 |
Externally published | Yes |
Keywords
- Africa
- Endemic
- Global health
- HHV-8
- HIV-negative
- Human herpesvirus-8
- KS
- KSHV
- Kaposi sarcoma
- Pediatric oncology
ASJC Scopus subject areas
- Epidemiology
- Oncology
- Cancer Research
- Infectious Diseases