Divergent clinical presentations and outcomes among children and adolescents with Kaposi sarcoma in Malawi and Tanzania

• Published in: HIV medicine 2023-06, Vol.24 (6), p.664-675
• Main Authors: Campbell, Liane R., Silverstein, Allison, Peckham‐Gregory, Erin, Kamiyango, William, Villiera, Jimmy, McAtee, Casey L., Bacha, Jason M., Kovarik, Carrie L., Mehta, Parth S., Chanroo, Toni, Kapesa, Asulwisye, Malingoti, Beatrice, Mzikamanda, Rizine, Ozuah, Nmazuo W., Allen, Carl E., Scheurer, Michael E., El‐Mallawany, Nader K.
• Format: Article
• Language: English
• Subjects: Adolescent; Anemia; CD4 antigen; Child; Children; Classification; Edema; Female; Health services; HIV; HIV Infections - drug therapy; HIV/AIDS; Human immunodeficiency virus; Humans; Kaposi sarcoma; Kaposis sarcoma; Kasabach-Merritt Syndrome; Malawi - epidemiology; Male; Patients; Pediatrics; Phenotypes; Sarcoma; Sarcoma, Kaposi - epidemiology; sub‐Saharan Africa; Survival; Tanzania - epidemiology; Thrombocytopenia
• ISSN: 1464-2662; 1468-1293
• DOI: 10.1111/hiv.13455

Objectives The Kaposi sarcoma (KS) T0 versus T1 staging classification does not address the unique clinical features of paediatric KS in human gammaherpesvirus 8 (HHV‐8) endemic regions of Africa. This study seeks to define patterns of childhood KS using a paediatric‐specific approach. Methods The Lilongwe paediatric KS staging classification categorizes disease based on clinical phenotype: stage 1 = mild/moderate KS limited to cutaneous/oral involvement, stage 2 = primarily lymphadenopathic disease, stage 3 = woody edema KS, stage 4 = visceral and/or severe/disseminated mucocutaneous disease. Characteristics and outcomes were evaluated from paediatric referral centres in Lilongwe, Malawi, and Mbeya, Tanzania. Results Among 171 patients, the median age was 9.3 years, 37% (n = 63) were female, and 87% (n = 149) had HIV. Breakdown by stage was as follows: 18% (n = 31) stage 1, 33% (n = 56) stage 2, 19% (n = 33) stage 3, and 30% (n = 51) stage 4. Age (younger stage 2 and older stage 3), severe CD4 count suppression (lower CD4 for stages 1 and 4), and presence of severe anaemia and thrombocytopenia (worse for stages 2 and 4) differed across stages. Estimated 2‐year event‐free survival/progression‐free survival/overall survival by stage was as follows: stage 1, 81%/81%/87%; stage 2, 50%/50%/63%; stage 3, 24%/49%/81%; and stage 4, 29%/34%/54%. Sub‐analysis of stage 2 lymphadenopathic KS demonstrated superior long‐term 6‐year event‐free survival of 70% (95% confidence interval [CI] 49–83) for younger children (aged