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Infect Agent Cancer. 2018 Nov 9;13:33. doi: 10.1186/s13027-018-0207-4. eCollection 2018.

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

Author information

1
1Baylor College of Medicine, Department of Pediatrics, Section of Hematology-Oncology, Houston, TX USA.
2
Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Global HOPE (Hematology-Oncology Pediatric Excellence), 1102 Bates Street, Feigin Tower, Suite 1025.16, Houston, TX 77030 USA.
3
Baylor College of Medicine Children's Foundation Malawi, Lilongwe, Malawi.
4
4Kamuzu Central Hospital, Lilongwe, Malawi.
5
5Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, NC USA.
6
6University of Pennsylvania, Philadelphia, PA USA.
7
7Center for Innovation, Quality, Effectiveness and Safety (IQuESt), Michael E. DeBakey VA Medical Center, Houston, TX USA.
8
8Baylor College of Medicine, Department of Medicine, Section of Infectious Diseases, Houston, TX USA.

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.

KEYWORDS:

Africa; Endemic; Global health; HHV-8; HIV-negative; Human herpesvirus-8; KS; KSHV; Kaposi sarcoma; Pediatric oncology

Conflict of interest statement

Ethics committee approval was obtained from the Malawi National Health Science Research Committee and Baylor College of Medicine Institutional Review Board for Human Subjects Research. Due to the retrospective nature of this study, a waiver of consent was obtained. All data were anonymized and de-identified prior to analysis to maintain strict protection of confidentiality.Not applicable.The authors have no competing interests to declare.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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