Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease Public Deposited

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  • Allen, Carl E
  • Mehta, Parth S
  • Dittmer, Dirk P
  • McAtee, Casey L
  • Legarreta, Alejandra
  • Martin, Stephen C
  • Villiera, Jimmy
  • El-Mallawany, Nader K
  • Kamiyango, William
  • Chiao, Elizabeth Y
  • Kazembe, Peter N
  • Kovarik, Carrie L
  • Peckham-Gregory, Erin C
  • Ozuah, Nmazuo W
  • Scheurer, Michael E
  • 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.
Date of publication
  • doi:10.1186/s13027-018-0207-4
Resource type
  • Article
Rights holder
  • The Author(s).
  • English
Bibliographic citation
  • Infectious Agents and Cancer. 2018 Nov 09;13(1):33
  • BioMed Central

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