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

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  • El-Mallawany, Nader K
    • Other Affiliation: Baylor College of Medicine
  • Villiera, Jimmy
    • Other Affiliation: Baylor College of Medicine
  • Kamiyango, William
    • Other Affiliation: Baylor College of Medicine
  • Peckham-Gregory, Erin C
    • Other Affiliation: Baylor College of Medicine
  • Scheurer, Michael E
    • Other Affiliation: Baylor College of Medicine
  • Allen, Carl E
    • Other Affiliation: Baylor College of Medicine
  • McAtee, Casey L
    • Other Affiliation: Baylor College of Medicine
  • Legarreta, Alejandra
    • Other Affiliation: Baylor College of Medicine
  • Dittmer, Dirk P
    • Affiliation: N.C. Cancer Hospital, UNC Lineberger Comprehensive Cancer Center
  • Kovarik, Carrie L
    • Other Affiliation: University of Pennsylvania
  • Chiao, Elizabeth Y
    • Other Affiliation: Michael E. DeBakey VA Medical Center
  • Martin, Stephen C
    • Other Affiliation: Baylor College of Medicine
  • Ozuah, Nmazuo W
    • Other Affiliation: Baylor College of Medicine
  • Mehta, Parth S
    • Other Affiliation: Baylor College of Medicine
  • Kazembe, Peter N
    • Other Affiliation: Baylor College of Medicine
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.
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  • Article
Rights statement
  • In Copyright
Rights holder
  • The Author(s).
Journal title
  • Infectious Agents and Cancer
Journal volume
  • 13
Journal issue
  • 1
Language
  • English
Bibliographic citation
  • Infectious Agents and Cancer. 2018 Nov 09;13(1):33
Publisher
  • BioMed Central

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